Zmіshana of illnesses with the resulting tissue є to rare illnesses, which are characterized by an overnight appearance of manifestations of systemic red blood vessels, systemic scleroderma, polymyositis or dermatomyositis and rheumatoid circulatory arthritis with rheumatoid arthritis Characterized by the development of brushes, the Raynaud's phenomenon, polyarthralgia, fiery myopathy, hypotonia and loss of function of the leg. DIAGNOSIS OF RUNTUNG On the analysis of clinical picture of the disease and antibodies to RNP for the duration of antibodies, characteristic of the majority of autoimmune diseases. Likuvannya is similar to this in case of systemic red blood pressure and the transfer of glucocorticoid impairment at an average and severe degree of impairment.

Zmіshane ailment with a resultant fabric (SZST) is seen in all sorts of people, among the representatives of the common races. The maximum of seizure disease is on an adult's age and another decade of life.

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Klіnіchnі show the mischievous infestation with the good fabric

Raynaud's phenomenon can show ailments. Often, the first manifestation of a small discomfort with the resulting tissue can nagaduvati an ear of systemic red blood vessels, scleroderma, rheumatoid arthritis, polymyositis or dermatomyositis. However, it is directly related to the nature of the cob manifestations of ailments, sickness is more severe until the progression and expansion due to the negative nature of the clinical manifestations.

Most often it is the swelling of the hands, especially the fingers, the result is the stench of sausages. Shkіri nagaduyut winks like this when you have a dermatomyosis. The reduction of the disease, similar to those in dermatomyositis, as well as ischemic necrosis and the appearance of the fingers in the fingers, develop earlier.

Bagato patients skary on polyarthralgia, 75% may be obvious signs of arthritis. For example, arthritis does not lead to anatomical changes, protects against erosions and deformities, such as in rheumatoid arthritis. Often the weakness of the proximal ligaments is promoted, as a result of the disease, and without it.

Efficiency nirok mіsce in about 10% of ailments and is often unaffected, however, in some people, it can lead to acceleration and death. With a loss of tissue loss, more often than not, after a loss of tissue loss, sensory neuropathy of the tricycle nerve develops.

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Diagnostics of the malignant disease with the resulting fabric

Zmіshane ailments of the resulting fabrics can be suspected in all ailments, who suffer from VKV, scleroderma, polymyositis or RA, with the development of additional clinical manifestations. Beforehand, it is necessary to carry out further monitoring for the presence of antinuclear antibodies (ARA), antibodies to the extracted nuclear antigen and RNP. If the results are discarded and will be indicative of a possible SZST (for example, there is an even higher titer of antibodies to RNA) ) -synthetase), anti-ribonuclease-resistant component of the extracted nuclear antigen (Sm) and sub-spiral DNA. The plan of those who have been asked to find out about the obvious symptoms of defective organ systems are: myositis, the reduction of niqs and the reduction in the number of specific methods of diagnostics (zokrema, MRI, electromyography, biopsies).

Bagato patients may have high titers (often> 1: 1000) of antinuclear antibodies, which can be detected by the fluorescence method. Anti-titer to the extracted nuclear antigen should be present in a very high titer (> 1: 100,000). Characterized by the presence of antibodies to RNP, todi as antibodies to the Sm-component of the extracted nuclear antigen on the day.

Forecast of the decline in the amount of money

The ten-fold vision is 80%, the prognosis lies at the stage of symptomatology. The main causes of death are Leheneva hypertension, nirkova deficiency, myocardial infarction, perforation of the intestine, desemination of infections, hemorrhage in the brain. For children, children can have a trivial remission without any kind of abuse.

It is not uncommon and more susceptible to the use of wicked disease in the case of systemic worms. Most of the patients, who may be ill of the middle and severe stage of turnover, are susceptible to glucocorticoid abuse, especially when it is too early to finish it. The absorption of the lung step is successfully controlled by salicylates, other non-steroidal anti-pyretic drugs, antimalarial drugs, in some cases - low doses of glucocorticoids. Variations in organs and systems reduce the signs of glucocorticoids at high doses (for example, prednisolone at a dose of 1 mg / kg 1 time per dose, orally) or immunosuppressants. The development of systemic sclerosis is carried out on a regular basis.

As for Zmishane

Zmіshane ailment with a lucrative fabric (SZVT)- a free clinical syndrome of systemic lesions with a fiery tissue and a fiery character, which manifests itself as a symptom of SSS, polymyositis (dermatomyositis), VKB, antihyl to rosular nuclear ribonucleoprotein); the prognosis is friendly, there is a low ailment, as a sign of a syndrome that is developing.

SZST was described by G. G. Sharp and sp_vavt. yak free "syndrome of rheumatic fever ailments". Unimportant to those who in the coming rocky bulo are aware of a lot of caution in the winter lands, the day of the SZST is still not open, since there is no unambiguous evidence - why is the self-consistent nosological form of the first ...

What provokuє Zmishan

In the development of ailments, they play the role of a free deterioration of immunity, which appears to be trivial in the development of antibodies to RNP, hypergammaglobuli not, hypocomplements in the immune system. Deposits of TgG, IgM and complement appear in the layers of blood-bearing vessels of the mucous membranes, glomeruli of nirka and dermoe-dermal stick of the dermis, and in the tissues of the liver, lymphoid and plasma infiltration. Installed changes in immunoregulatory functions of Tlimphocytes. Particularly in the pathogenesis of SZST є development of proliferative processes in the internal and middle sheaths of the great Sudins with a series of legacy hypertension and the lowest manifestations of the disease.

Symptoms of malnutrition with the resulting tissue

It is indicated in the name of SZST, the class of ailments is indicated by such signs of SJS, such as Raynaud's syndrome, swelling of the hands and hypokinesis in the duct, as well as symptoms of polymyositis and VKV in those with polyarticular and recurrent

Raynaud's syndrome- one of the most common signs. Zokrema, behind our materials, Raynaud's sydrome is of significance for all ailments from the designation of the SZVT. Raynaud's syndrome is not only partial, it is not very frequent and early signs of ailments, proteases on the form of SSV are against m'yakshe, often on a two-phase stalk, but the development of ischemic necrosis in some cases is in some cases.

Raynaud's syndrome in FDTS, as a rule, is superfluous to become thickened brushes right up to the development of a "sausage-like" shape of fingers;

Douzhe free myazova symptoms- in clinical pictures, ailments relieve pain and muscle weakness in the proximal diseases of the disease for fast polygons due to the infusion of medium doses of corticosteroid therapy. Instead of meat enzymes (creatine phosphokinase, aldolase), it grows smartly and quickly normalizes for hormone therapy. Occasionally, it is characteristic of dermatomyositis that there is a reduction of the skin over the swelling of the fingers, heliotropic formation of the pockets, teleangiectasis along the edge of the nail bed.

Svoєrіdna suglobova symptomatology... Patients who suffer from a pathological process of lobes are practically susceptible to developing in all those ailments, the head rank in those who suffer from severe polyarthralgia, and in 2/3 of those who suffer from polyarthritis (non-erosive, as a rule, undeformable ones), they want to develop ... It is characteristic of the processes of great slopes of the order from the change of other slopes of penzliv, like from the BKV. It is not difficult to describe inadvertently from the RA erosive and destructive wrinkles in the nodules of the hands. Similar snorts were spoken by the ailments in our institute.

Hіpokіnezіya stravohohod to develop in ailments and is tied to the relativity not only of radiological dosages, but of manometric ones, the failure of the collapse of the traumatic channel in the region is very close to such a step, as in the case of STS.

Exercise of serous sheaths Sposter not so often, as with VKV, prote at SSTD, bilateral pleural pleurisy and pericarditis are described. Significantly more often is the loss of a pathological process of the disease (ventilation damage, decrease life, and with roentgenologic dosage - improvement and deformity of the Legeny baby). At the same hour, Legenev's symptomatology in the vicinity of ailments can be seen as the main role, appearing as an overgrowth and / or symptomatology of Legendary hypertension.

The specialty of the SZST nirok battle(according to the literature, in 10-15% of the ailments), but in the ailments, in those who have a protein, hematuria, or morphological changes in biopsy nirok, call to mean a good interruption. Vinyatkovo rarely spares the development of nephrotic syndrome. For example, according to the data of the clinic, the level of nirok is assigned to 2 out of 21 sick people on the SZST.

Cerebrovasculitis is also rarely diagnosed, polyneuropathy is mildly reversed, which is not a sign in the SZST class.

Among the out-of-the-way clinical manifestations of ailments, there is a growing degree of differentiation. hot reaction and lymphadenopathy(in 14 to 21 patients) and before splenomegaly and hepatomegaly.

It is not common for SZST to develop Segren's syndrome, or even a good course, like for VKV.

Diagnostics of Zmіshany illusion with the resulting fabric

• Laboratory data

General laboratory data for nonspecific SZST. Approximately in half of the ailments in the active phase of the ailment, it means less hypochromic anemia and a tendency to leukopenia, in all of them - accelerated SHOE. However, serological dosages are typical for ailments on the development of antinuclear factor (ANF) with mottled type of immunofluorescence.

In ailments on SZST, a high titer of antibodies to nuclear ribonucleoprotein (RNP) - one of the most important nuclear antigens, sensitive to the infusion of ribonuclease and trypsin - appears. Yak viyavilosya, the same antibodies to the RNP and the other developed nuclear antigens add to the nuclear type of immunofluorescence. In fact, the serological features of the order due to the meanings of the clinical features of the classic nosological forms have become the reason for the appearance of the SZST syndrome.

In addition, it is often referred to as gipsrhammaglobulin, not rarely overwhelming, as well as the regions of the Russian Federation. At the same time, the SZST is especially characterized by the intensity and turnover of the cichlids, regardless of the amount of activity of the pathological process. At the same hour, in the active phase of the ailment, circulating immune complexes do not appear so seldom, and it is light hypocomplements.

Zm_shanyi zhvoryuvannya sluchnuyu fabric

Characterized by the temporal efficacy of corticosteroids at medium and low doses for each type of SJS.

Oscillations in the rest of the rock are indicated by the tendency to the development of nephropathy and legeny hypertension, ailments with many key signs will require an hour of consuming large doses of corticosteroids and cytostatic drugs.

The prognosis of the ailment is rather daunting, the problem of death is described, but it is very important to diagnose if there is a deficiency or a legenevium of hypertension.

Until some of the likars go to death, as long as you have a chance to get sick with a good cloth.

Rheumatologist

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Diffuse inferiority with the resulting fabric

Diffuse discomfiture of tissue (DZST) or colagenosis (term, which is historically meaningful) is a group of discomfort, characterized by systemic immune-ignition damage from the resultant tissue of the elderly. A whole group, ale from a nosological understanding, at the connection with the same term of the year, it would mean some nosological forms.

DZST ob'єdnuyut to fill up a bagato zhvoryuvan. Most widened VKV, SSD and DM. Before the whole group, the disease can also be recognized as an ORL, as it is traditionally described in the distribution of the disease of the heart-vessel system. It has been reported that the DZST can lead to a breakdown in immune homeostasis, which can develop in the development of autoimmune processes, tobto. reactions of the immune system, which are supervised by the statements of antigen or sensitized lymphocytes, which are conjugated against antigens of the vascular organism.

In the basis of autoimmune disorders there is an immunoregulatory imbalance, which rotates in the reduced suppressive and strong helper activity of T-lymphocytes with a further activation of B-lymphocyte products of such hypothermic

I have a number of extra rice to enjoy DZST:

Specificity of pathogenesis - impairment of immune homeostasis in viglyadi uncontrolled viralization of autoantitilation and establishment of immune complexes "antigen-antigen", which circulates in the blood and is fixed in tissues with a more advanced developmental reaction of heavy

A selection of morphological changes (fibrinous changes of the main speech in the resulting tissue, vasculature, lymphoid and plasma infiltration and in.);

Chronic overload with periods of fire and remission;

Tightening for non-specific injections (infectious disease, insolation, vaccination, etc.);

the systematic nature of the battle (skin, loam, serous shell, nirka, heart, legends);

Licuvalny effect of immunosuppressive drugs (glucocorticoids, cytostatic drugs).

All sickness, which is included in the whole group, is recognized by the basic and morphological signs, which in the specific skin type is due to the exact nosological diagnosis.

In a wide variety of representations, diagnostic poses during VKV, STS and DM.

SYSTEM OF CHERVON VOVCHENKA

Systemic chervoniy vovchak (VKV) - systemic autoimmune disease, as a result of a young person (especially in women) and develop on the genetically intelligent lack of immunity of immunoregulatory processes, to bring antibodies to uncontrolled production of antibodies to the power cells of this chronic immune complex A. ... The presence of the disease in the polyagus in the immune-fired tissue, microcirculatory bed, shkiri, suglobiv that internal organs, at the same time, they are responsible for visceral injuries, so that the prognosis of ailments starts.

Occurrence on VKV becomes 4 to 25 per 100 yew. population. Infection most often develops among women of the pre-epoch. Every hour of vaginosity in the psychoanalytic period is signi fi cantly grows up sharply. Women stay on VKV 8-10 times, often per person. The peak of convalescence will come up to 15-25 years. In children, children with ailments of females and lads decrease and become 3: 1. Mortality during VKV is in great numbers, but not among the population. Among the choloviks, the protesters are as important as those of women.

VKV to follow up to genetically determined illness: before the diagnosis, carried out in the population, it was shown that the condition before VCB was linked to the singing genes of the II class of hystosumity (HLA), genetically accumulating the deficiency of complementary factors and components of the FNP-α).

Ethiology

A specific ethological factor in case of VKV is not set up, but a number of key symptoms (cytopenic syndrome, erythema and enanthema) and the regularity of the development of ailments are allowed by the association of VKV due to illnesses viral ethology... In a given hour, the value of RNA-viruses is generated (mostly or latent viruses). The appearance of family diseases, often in the family of rheumatic or allergic ailments, and young people who suffer from immunity are allowed to think about the possible meaning of family-genetic schism.

Manifestations of VKV with a level of low nonspecific factors - insolation, nonspecific infection, the introduction of syvates, lykarsky zasob_v(zokrem, peripheral vasodilators from the group of hydralazines), as well as stress. VKV can read about abortion. All tsі danі allow viewing VKV as a multifactorial disease.

Pathogenesis

In addition, a virus is injected into the immune system, and possibly, anti-tier antibodies, on the basis of a decline in the rate of immunity, dysregulation of the immune response, so that it can lead to a hyperreactivity of the humoral immune system. In the organisms of ailments, the production of antibodies to the young tissues, cells and blood cells (including up to the older cells and DNA) is uncontrolled. It has been established that during VKV autoantitila is violated up to approximately forty more than two hundred potential antigenic cell components. The establishment of immune complexes and attachments to the organs and tissues (especially in the microvasculature) is necessary. Characteristic malignant immunoregulation defects, which are supervised by hyperproduction of cytokines (IL-6, IL-4 and IL-10). Then the processes are developing, connected with the eliminal fixed immune complexes, to produce the development of lysosomal enzymes, the development of the organs and tissues, and the development of the immune firing. In the process of burning and destruction of the resulting tissue, new antigens are generated, and the formation of antigens and the establishment of new immune complexes are triggered. With such a rank, the winery is viciously prickly, which will prevent the chronic interruption of the affliction.

Classification

At this time in our country, I am working on the classification of key options for the transition of the VKV, yak vrahovu:

The nature of the leak;

the activity of the pathological process;

Clinical and morphological characteristics of the affected organ systems. The nature of the sickness

The state of the art is characterized by a quick development of multiorgan changes (including an increase in the level of the central nervous system) and high immunological activity.

Pidgostry overrun: at the onset of illness, the main symptoms are found, nonspecific lesions of the shkiri and suglobiv. Poisoning against the disease is similar, with periodic sharpening and development of polyorganic debris stretching 2-3 times at the time of the first symptoms.

Chronic stretching is characterized by trivial problems of the same number of signs: recurrent polyarthritis, discoidal syndrome, Raynaud's syndrome, Verlhof's syndrome and Sjogren's syndrome. Multiple organ patches can grow up to 5-10 ailments.

Phase and step of activity to the process:

Active (temporal activity - ІІІ, pomirna - ІІ, minimal - I);

Inactive (remis_ya).

Clinical and morphological characteristics of strikes:

Shkiri (symptom of "blizzard", capilarity, exudative eritema, purpura, discoid vovchak and in);

Suglobiv (arthralgia, gostry, pidgostria and chronic polyarthritis);

Serous membranes (polyserositis - pleurisy, pericarditis and perespliv);

Sertsya (myocarditis, endocarditis, lack of mitral valve);

Lungs (hospitality and chronic pneumonitis, pneumosclerosis);

Nirok (lupus-nephritis of nephrotic chi zm_shany type, sečovy syndrome);

Nervous system (meningoencephalopolyradiculoneuritis, polyneuritis).

With chronic overload of ailments, 20-30% of ailments develop the so-called antiphospholipid syndrome, presentation of clinical and laboratory symptom complex, including venous thrombosis, arterial thrombosis, development of organ obstetric pathology, thrombosis. A characteristic immunological sign is the establishment of antiphosphates, which react with phospholipids and phospholipids and phospholipids (more details about antiphospholipid syndrome will be said later).

There are also three stages of activity of the pathological process, which characterize the turnover of the potential circulating immuno-inflammatory ear and the initial specificity of the treatment of a specific skin disease. The activity of the emergence of grave illnesses, as a result of which there is a rise in the likelihood of non-gated wickedness, potentially insecure ailment.

key picture

The clinical picture of ailments is superbly versatile, it is tied to the multiplicity of organisms and systems, the nature of the interruption, the phase and stage of activity of the ignition process.

To receive information, at the shop you can submit:

About a variant of a corncob;

the nature of the ailment;

the level of involvement in the pathological process of quiet organs of systems;

The treatment was carried out earlier, and the efficiency of the procedure can be accelerated.

Options for a cob of ailments can be nyr_znomantn_shim. Most of them are represented by a number of young syndromes. Monosymptomatic ear, as a rule, is not characteristic. Have a zvyazku z tsim poodennya about zhvoryuvannya VKV vinyk zviyavlennya from a sick person such a day. And here we see the diagnostic value of quiet syndromes.

In the early period of VKV, the most common are the syndromes of urazhennya loam, shkiri and serous shells, as well as fever. Such a rank, the most susceptible to VKV will be today:

Likhomanki, polyarthritis and trophic porcine shkiri (zokrema, hair loss - alopecia);

Polyarthritis, fever and pleurisy (pleurisy);

Likhomanki, trophic porosheni shkіri and urazhennya pleurisy.

Diagnostic significance of ciches by a significant world grows, as the battle of the cough is represented by the erythema, but at the cob period of the sickness it is reduced to 25% of the problems. The protection of the surroundings does not lower the diagnostic value of the change in food.

The malosymptomatic ear of ailment is not characteristic, although the debut of VKV from the appearance of massive swellings as a result of the development of the ear of diffuse glomerulonephritis (lupus nephritis) of the nephrotic abnormal type is less significant.

Induction to a pathological process of other organs is manifested by symptoms of a fiery lesion (arthritis, myocarditis, pericarditis, pneumonitis, glomerulonephritis, polyneuritis, etc.).

Seeing about the treatment, which was carried out earlier, it is possible to judge:

About its optimum;

About the state of ailment, and the level of activity in the process (cob doses of glucocorticoids, the triviality of the intake of ailments, including the intake of cytostatics before the complex of cytostatics in case of swelling of nephritic disorders, high activity)

About accelerated glucocorticoid and cytostatic treatment.

On the first stage, it is possible to diagnose a disease with a trivial outbreak of ailment, but at the beginning of the debut, the diagnosis can be made on further stages of the disease.

It is possible to reject the impossibility of giving, to testify about the destruction of organs and the steps of their functional deficiencies.

The dissection of the supporting-mucous apparatus manifestus with polyarthritis, as well as symmetrical lesions of the other lobes of the cyst (proximal mid-phalangeal, p'acphalangeal, interstitial) and great lobes. When open-minded classical pictures become ill, they start defining the slopes, zoomed with a periarticular embryo. With a stretch of ailments, deformation of other slopes deforms. Loose wrinkles can supervise the fight against mucus in vigorous diffuse mialgia, and even more rarely - true PM because of the thick and weak mucus. Inodi strikes are represented by lichen arthralgias.

The cutting of the shk_rnogo cover is so very often, like the slopes. Naybіlsh types іrіthematoznі visipannya on faces in the areaі of the arches і of the back of the nose ("blizzard"). Ignite hanging on the noses and cheeks, which repeat the "blizzard" outlines, represented by different options:

Sudden (vascular) "blizzard" - unstable, pulsating, spilling pockets of shkiri with cyanotic appearance near the middle zone of the face,

how to do it on the basis of different factors (insolation, winter, cold) or hygiene;

... "Blizzard" type of centralized eritemi (the change of the school is localized only in the region).

It is possible to develop a "blizzard" for discos - erythematous plaques, which develop, with keratical damage and a further development of the atrophy of the skin of the face, images and tuluba. Nareshty, in some of the ailments, it means a nonspecific exudative erythema in the school of children and breast cells, as well as signs of photodermatosis in the most critical parts of the body.

Before the shkіrny cover, carry out the capillary - a punctate hemorrhagic visip on the pads of the fingers, nіgtovy beds and lobes. Shkіrnі strikes can unite with enanthema on a firm pіdnebіnnі. On the mucous membrane of the mouth, or the nasopharyngeal dilation, it is possible to create painless movements.

Cutting of the serous sheaths of vinicum in 90% of ailments (the classic diagnosis is dermatitis, arthritis, polyserositis). Especially often, there is a lesion of the pleurisy and the pericardium, and more - the neoplasm. Symptoms of pleurisy and pericarditis are described in in front of them To that, far away will be reinsured without their specialness during VKV:

Most often, dry pleurisy and pericarditis;

In case of vipitnyh forms, the amount of exudate is small;

Cutting of the serous membranes in a short time, і let them diagnose retrospectively when pleuropericardial adhesions are revealed, or a draining of the costal, mid-area and mediastinal pleurisy in case of radiological diagnosis;

This leads to a trend towards the development of sticky processes (increasing growth and development of gray empty ones).

For the VKV, the urazhennya of the heart-vascular system is even more characteristic, but in the winter stages, the ailments are overwhelmed.

Most often, pericarditis appears, stopping until relapse. Significantly more often, now it was mentioned earlier, it means that the endocardium in viglyadi warty endocarditis (vvchakovy endocarditis) on the stools of the mitral, aortic abdominal valve. With a trivial interruption to the process on another stage, the joke can show signs of a lack of an external valve (signs of a stenosis of the opening, as a rule, visible).

Vognischevic myocarditis is practically nicoli not restructuring, ale diffuse lesions, especially in severe interruption, supravital symptoms (div. "Myocarditis").

Disorder of the vessels can be manifested by Raynaud's syndrome, for which is characteristic of the arterial blood loss of the hands, which spasmodically develops, and (abo) feet, which is caused by the cold, or hvilyuvannya. For an hour I will attack by means of paresthesia; a shkira of fingers is old and (abo) cyanotic, fingers are cold. More importantly, the measurement of the II-V fingers of the hands and feet, before the second distal fingers of the hand (nose, vuh, pidboriddya and in.).

The astonishing light can be made to the main illnesses and secondary infections. The ignition process in the lungs (pneumonitis) is contrary to the state of the art and in the months and manifests signs of the syndrome of inflammation of the lung tissue, similar to those in pneumonia. The peculiarity of the process of clearing is in the diagnosis of an unproductive cough in the short term. The second variant of the lesion is chronic interstitial changes (firing of the perivascular, peribronchial and interdigital tissue), which can be used to develop more and more progressively worse lungs and x-rays. The characteristic physical data is practically everywhere, so it is practically unwise to judge about such a lesion on another stage of a diagnostic joke.

The dissection of the shKT, slack, is represented by sub-active signs, manifesting the first stage. With a physical condition, there is an unimportant soreness in the epigastric disease and in the projection of the disease, as well as signs of stomatitis. Hepatitis develops in a number of patients: it means an increase in liver soreness.

Most often, with VKV, the diagnosis is nirok (vovchakovy glomerulonephritis or lupus-nephritis), as a result of the evolu- tion of which the share of the ailing person can be found. The breakdown of nirok at VKV can be contradicted by the viglyadі of іznіh options, to that the tribute to the unprecedented covering of the sick can be widely varіyuvati. In case of isolated changes of slaughter, the siege does not appear before the hour of physical release. With glomerulonephritis, which is against nephrotic syndrome, there are massive swellings and uncommon arterial hypertension. When formulated chronic nephritis From the post-AH, there is an increase in the lingering little one and the accent of the second tone in the other mid-ribs, the right-handed from the breastbone.

Autoimmune thrombocytopenia (Verlhof's syndrome) manifests itself as typical visceral growths in hemorrhagic beaches of a small size on the inner surface of the skin, the chest and abdomen, as well as on the mucous membranes. For minor injuries (for example, for extractions of a tooth), bleeding occurs. Nose bleeding is one that swells profuse character and produces anemia. Shkirnі bloodshed can be maternally lowered: blue-green, tempest or zhovte. Frequently, VKV does not have a history of Werlhof's syndrome without any typical clinical symptoms.

Smacking nervous system is rotated by the growth of the world, the pathological process may be more likely to occur. Ailments present skargi for a head bile of a migraine character. In some cases the ships are attacked. May damage to cerebral blood flow to the development of an insult. When a person is ill, there are signs of polyneuritis with impaired sensitivity, ailments along the nerve stubs, decreased tendon reflexes and paresthesias. Organ cerebral syndrome is characterized by emotional lability, depression episodes, memory loss and bewilderment.

The division of the reticuloendothelial system is represented by an early symptom of generalization to the process - polyadenopathy (the growth of all groups of lymphatic universities, but not reaching a significant step), as well as, as a rule, with the help of a sage.

The damage to the body is manifested with dry keratoconjunctivitis, with pathological changes in lazy valleys and impaired functions. Dryness of the eyes can lead to the development of conjunctivitis, erosion of the cornea, or keratitis with damage to the eye.

With antiphospholipid syndrome, venous disorders can occur (in the glib veins of the lower symptoms with repeated thromboembolism of the lung artery) and arterial (in the arteries of the brain, which can lead to thrombosis and transient ischemic attacks). Restructuring of the valve wadi of the heart, internal heart thrombosis, and the reduction of the myxoma of the heart, and thrombosis of the coronary arteries with the development of IM. Disorders of children with antiphospholipid syndrome, aggravated disease, ale nayposhirenish їkh - often livedo (Livedo reticularis).

In this rank, pіslya another Etap obstezhennya viyavlyayut polіorgannіst urazhen, wherein їh stupіn duzhe rіzna: od Led River klіnіchno pomіtnih (subklіnіchnih) to virazhenih scho perevazhayut over іnshimi scho stvoryuє peredumovi for dіagnostichnih pomilok - іnterpretatsії Tsikh Change log yak Find our samostіynih zahvoryuvan (napriklad, glomerulonephritis ). myocarditis, arthritis).

The third stage of diagnostics joke with VKV, the value is even greater, spikes:

Additional help to establish residual diagnosis;

Demonstration of the turnover of immune damage and the level of damage to internal organs;

Allows for the number of steps in the pathological (Vovchak) process.

At the third stage, the most significant is the development of laboratory blood tests. Virіznyayut two groups of indicators.

Indicators that can be directly diagnosed (to indicate the turn of immunological damage):

LE-cells (cells of the chervony vovchaku) - mature neutrophils, which phagocytose the nuclear cells of the blood cells, which grow up before the ANF.

ANF ​​is a heterogeneous population of autoantitils, which react with the small components of the cell nucleus and circulate in the blood (95% of ailments show up at a titer of 1:32 and in food). The presence of the ANF in the overwhelming majority of cases is to indicate against the diagnosis of VKV.

ANA - antibodies to native (tobto to a whole molecule) DNA. Increasing the concentration of the core due to the activity of the disease and the development of vovchak jade. Їх appear in 50-90% of ailments.

Antibody to Sm-nuclear antigen (anti-Sm) is highly specific for BKV. Antibody to Ro / La ribonucleoprotein is used specific for VKV (by immunofluorescence method it is detected in 30% of patients, by hemaglutination method - in 20% of patients).

The phenomenon of “rosettes” is a change in the nucleus (hematoxylinovy ​​tilt), which can lie near the tissues, oozing with leukocytes.

Diagnosis of antiphospholipid syndrome in VCV is based on the presence of additional blood clotting anticoagulants - specific antibodies to phospholipids, which appear in case of significant blood loss due to additional functional antihypertensive tests. The term "vovchakovy anticoagulant" is not wrong, so the main clinical sign of the presence of high-grade antibodies is thrombosis, not bleeding. The assigned antiphosphates show up in the so-called primary antiphospholipid syndrome - self-inflicted illness, in cases of thrombosis, obstetric pathology, thrombocytopenia, and, often, lyvedo and autoimmune hemolytic hemolysis.

Nonspecific acute phase indicators, up to which include:

Dysproteinemia with the advancement of α 2 - and γ-globulin;

Vyavlennya SRL;

Increased concentration of fibrinogen;

Pidvischennya SHOE.

In case of swelling, low-titer striations, one can know RF - antibodies to the Fc-fragment of IgG.

When peripheral blood is advanced, it is possible to develop leukopenia (1-1.2x109 / l) from a leukocyte formula to the young forms and mylocytes in some other lymphocytes (5-10% lymphocytes). Hypochromic anemia can be reduced, in some cases - hemolytic anemia, which can be superimposed on zhovtyanitsa, reticulocytosis and positive Coombs breakdown. Inodi restructuring of thrombocytopenia in patients with Werlhof syndrome.

For striking nirok characteristic wrinkles in the slash, as it is possible to classify in such a way (I.E. Tareeva, 1983):

Subclinical proteinuria (instead of a bottle in a section of 0.5 g / dob, often - in a small leukocyturia and an erythrocytosis);

Proteinuria is more pronounced, which serves as a virase of nephrotic syndrome, which is superficial and active or active vovchakovy nephritis.

Outside of the temple, proteinuria (yak, for example, with amyloidosis) develops rapidly. I mean I will have hemorrhage. Leukocyturia can be a bit like a vovchak ignition process in nirkas, as well as the result of an unremitting secondhand infection of every day.

With punctate biopsies, nonspecific mesangiomembranous changes, often with a fibroplastic component, appear. Characteristic vvazhayut:

Evidence in drugs lying in nirkovy tissue, changing nuclei (hematoxylin cells);

Capillary membranes of the glomeruli at the viglyadі of the darting loops;

Placement on the basal membrane of glomeruli in fibrin and immune complexes in electronic deposits.

The following morphological types of nephritis are seen for the classification of the WHO:

Class I - the duration of the change.

Class II - mesangial type;

Class III - middle proliferative type;

Class IV - diffuse proliferative type;

Class V - membranous type;

Class VI - chronic glomerulosclerosis.

When X-ray dosage is detected:

Snakes in the slopes (in case of a lump syndrome - epiphyseal osteoporosis in the folds of the wrists and inter-wrist slopes, in case of chronic arthritis and deformities - ringing of the swelling line with pidvivikha);

Zmіni in legends with the development of pneumonia (with a trivial overload of ailments - disc-like atelectasis, improving the deformity of the legeny malunky in the presence of high diaphragm stagnation);

Zmini from the side of the heart with the development of Vovchakovo wadi or exudative pericarditis.

The EKG allows the creation of nonspecific changes in the end portion of the clown complex (tooth T that segment ST), analogous described earlier for myocarditis and pericarditis.

CT and MRI of the brain reveal pathological changes in the lesions of the central nervous system.

When carrying out a diagnostic joke, it is also necessary to determine the steps of the activity of the vovchak process (Table 7-1).

Table 7-1. Criteria for the activity of a pathological process in systemic heart disease (Nasonova V.A., 1989)

Expansion of the table. 7-1

Diagnostics

In the case of a classical interruption, the VKV-diagnostics is simple and is based on the occurrence of a "blizzard", recurrent polyarthritis and polyserositis, which can become a key diagnostic process, so that the presence of an LEH-type diagnostics can be added. In addition, there are young people with ailments, ligaments from canopies, abortions, an ear of menstrual function, insolation and infectious diseases. It is much more foldable to establish a diagnosis in those types of diseases, especially when the diagnosis is carried out in the classics of diagnostics. In the current situation, additional diagnostics criteria, breaking up by the American Rheumatology Association (ARA) at 1982 p. she looked over at 1992 p. (Table 7-2).

Table 7-2. Diagnostic criteria for systemic heart disease (ARA)

The end of the table. 7-2

Diagnosis is reliable in case of certain criteria or more. As soon as there are fewer criteria, then the diagnosis of VKB summaries, and it is necessary to dynamically caution for the ailment. Such a symptom is not correct: we are susceptible to such ailments on glucocorticoids, some of the same symptoms can be associated with other symptoms (including paraneoplastic syndrome), in the case of some

Differential diagnosis

VKV Slid Differentsiyuvati z low zhvoryuvan. There is a great change of organs of the prosecutor's office and systems that have been acquired in pathological processes during VKV, there is an extensive list of ailments that can be diagnosed in a sick person. VKV can be of a greater world and the development of pathological conditions. It is especially often to trawl at the debut of an affliction, as well as during a dominant defeat of one or two organs (systems). For example, if a disease of pleurisy appears on the cob of ailment, it is possible to develop pleurisy of tuberculous etiology; Myocarditis can be treated as rheumatic and nonspecific. It is especially rich in pardon to let it go, when VKB debut with glomerulonephritis. In such cases, diagnose deprivation of glomerulonephritis.

VKV most often differentiates from ARF (rheumatism), IIE, chronic active hepatitis (CAH), hemorrhagic diseases (thrombocytopenic purpuri) and minor ailments from the DZST group.

The need for differential diagnostics for rheumatism in women, as a rule, in children and young people at the debut of becoming ill - with diagnosed arthritis and fever. Rheumatic arthritis develops as a result of a greater degree of symptoms, which is more severe than the lesions of great slopes and shvidkoplinny. Do not put pressure on the differential diagnostic value of the frontal infection control (angina), some of them can serve as a nonspecific factor, but the development of key signs of VKV. Diagnosis of rheumatism becomes reliable with a diagnosis of heart disease (rheumatic heart disease). Farther dynamically, caution is allowed to allow the defect of the heart to form, even if the mitral valve is lacking, if the lack of the mitral valve is formed, then it rotates slightly and does not supervise readily

hemodynamic damage. Mіtrala regurgіtatsіya swirls insignificant world. On the basis of VKV, at the stage of rheumatism, leukocytosis is considered. ANF ​​does not appear.

Differential diagnostics between VKV and RA is twisted in the cob stage of ailments, which is tied to a similar key picture: the winery is a symmetrical cut of other slopes of the brush, the process of getting new early scrapes is characteristic. Differential diagnosis is based on re-infection with RA in the slopes of the proliferative component, early development of the hypotrophy of the mucous membranes, which leads to the infection of the slopes, and the stiffness of the slopes. The erosion of the loamy surfaces with VKV is visible, ale є is a characteristic sign of RA. High titer of the Russian Federation of the rate of RA. When VKV yogo viyavlyayut rare at a low titer. Vinyatkovo folding differential diagnosis of VKV and visceral form of RA. Clarified diagnosis in both vapadas does not infuse into the nature of the lykuvannya (sign of glucocorticoids).

With CAH, there may be a systemic breakdown in patients with fever, arthritis, pleurisy, skin diseases and glomerulonephritis. It is possible to develop leukopenia, thrombocytopenia, LE-cellini and ANF. Per hour of carrying out differential diagnostics of the following:

HAG often develops in the middle;

In the anamnesis of ailments on CAH є cases of viral hepatitis transfers;

With CAH, there are changes in the structure and function of the liver (cytolic and cholestatic syndrome, signs of liver failure, hypersplenism, portal hypertension);

With VKV, the liver infection is not susceptible to or against mild hepatitis in patients with hepatitis (in addition to signs of cytolytic syndrome);

With CAH, there are signs of viral liver damage (anti-viral antigen and viral antigen).

In the case of primary IIE, there is a rapid heart failure (lack of the aortic chi-mitral valve), and antibacterial therapy is even more effective. LE-cells, antibodies to DNA, ANF, which, as a rule, are visible. With a timely bacteriological follow-up, the growth of pathogenic microfloria is revealed.

In case of thrombocytopenic purpura (idiopathic or symptomatic), there are frequent syndromes, which are diagnosed with VCV, types of laboratory signs (LE-cellini, ANF, anti-DNA to DNA) and a fever.

Naybilsh foldable differential diagnostics of the other incapacitated groups of DZST. Such stani, yak SSD and DM, can be used for mothers without sleeping rice from VKV. The situation is as simple as possible when the ANF is ill and LE-klitin, I want a lower title. The main differential diagnostic signs are that the internal organs are often affected (especially nirok) with VKV, the more severe nature of the disease with SSS and a clear myopathic syndrome with DM. In a number of vipadkiv, put the correct diagnosis, allowing for more trivial

a dynamic look for ailments. In some cases, there is a lot of money and rockets (especially in the case of a chronic VKV current due to a minimal level of activity).

The formulation of the open-ended clinical diagnosis of VKV can be found in all the headings, which have been introduced at the working classifications of discomfiture. Diagnosis is guilty of displaying:

The nature of the overload of the ailment (gostry, pidgostria, chronic), and in case of chronic overload (mono-oligosyndromic), it leads to provincial clinical syndrome;

activity of the process;

Clinical and morphological characteristics of the damage to the organs and systems due to the development of the stage of functional deficiency (for example, in case of lupus nephritis - the stage of normal deficiency, in case of myocarditis - in case of lack of heart disease, heart failure)

Application for lykuvannya (for example, glucocorticoids);

Uskladnennya lіkuvannya (yaksho stink є).

Likuvannya

Vrahoyuchi the pathogenesis of getting sick, we are sick on VKV it is recommended to complex pathogenetic treatment. Yogo zavdannya:

Immune inflammation and immunocomplex disorders (uncontrolled immune diseases);

Increased acceleration of immunosuppressive therapy;

Likuvannya accelerated, which is in the process of carrying out immunosuppressive therapy;

Injection on okremі, rapidly turning syndromes;

Visualization of the organ_ism of the CVC and antil.

In the first place, it is necessary to eliminate psycho-social stress, insolation, actively eliminate the suppression of infectious diseases, to live a low-fat diet with a high level of polyunsaturated fatty acids, calcium chain and D. Do not take a contraceptive because of the high amount of estrogen, the spite of the aggravation of the disease.

For suppression of immune inflammation and immunocomplex breakdowns in the case of VKV elimination, use the main immunosuppressors: glucocorticoid short-term, cytostatic drugs and aminochinolines. The triviality of the drug, the vibration of the drug, as well as the dosage, which can be accepted, starts:

by the level of activity;

the nature of the leak (state of the art);

A wide range of results up to the pathological process of internal organs;

The transfer of glucocorticoids or cytostatics, as well as the decrease in the rate of accelerated immunosuppressive therapy;

Contraindications to the news.

At the cob stages of ailments with minimal activity, the reduction in the clinical picture of glucocorticoid locks should be indicated at low doses (prednisolone at a dose of less than 10 mg for dobu). If you are guilty of being transferred to the dispensary oblast, if you are diagnosed with the first signs of aggravated illnesses of drugs, you can easily identify the use of glucocorticoids in the optimal dose.

In case of a chronic overload of ailments in case of overwhelming attacks of children with a stretch of bags, it is possible to use chloroquin (dose 0.25 g / dob) or hydroxychloroquin.

When there is a sign of high activity and generalization of the process from the study to new internal organs, it is necessary to safely switch to more effective immunosuppressive treatment with glucocorticoids: to indicate a dose of pre-dose of glucocorticoids. The triviality of taking high doses varies from 4 to 12 months. Decreasing the dose should be carried out step by step, with the help of retail clinical and laboratory control. Take a dose (5-10 mg per dose) for ailments, take a handful of rocks.

In such a rank, the main method of VKV treatment is glucocorticoid intake. When you are victorious, follow the offensive principles:

Fix a lichen lesion in the case of a confirmed diagnosis of VKV (if the drug is not consumed);

The dose of glucocorticoids may be sufficient to suppress the activity of the pathological process;

Likuvannya with an overdose dose should be carried out until a rotated clinical effect is reached (a reduced backbone, normalization of temperature, reduced laboratory parameters, positive dynamics of organ changes);

When the effect is reached, step by step go to the dose, which is ready;

Obov'yazkovyu є prophylaxis of accelerated treatment with glucocorticoids. To get overwhelmed bit by bit glucocorticosteroids stasis:

Calcium preparations (oral acid, calcium chloride, calcium and magnesium asparaginate);

Anabolic dose (metandinone at a dose of 5-10 mg);

Sechogіnnі (saluretics);

Hypotensive drugs (ingibitori ACE);

Take antacids.

For the development of severe acceleration, the following should be noted:

Antibiotics (for secondary infections);

Anti-tuberculosis drugs (for the development of tuberculosis, often - legacy localization);

Preparations insulinu, dyetichne kharchuvannya (for sugar diabetes);

Anti-fungal infection (for candidiasis);

Anti-rash treatment (with approved steroid treatment).

For an hour, the use of glucocorticoids determines the situation, if it is necessary to administer extra high doses of prednisolone (internally dribbled in a dose of 1000 mg for 30 minutes for three days):

Rise of the increase (splash) of activity to the process (III stages), unaffected, it would be better to use it;

resistance to doses, with the help of some earlier reached a positive effect;

Variations of organ changes (nephrotic syndrome, pneumonitis, generalization of vasculitis, cerebrovascular disease).

Pulse therapy is similar to the establishment of immune complexes for galvanizing the synthesis of antibodies to DNA. Reducing the concentration of residuals, with glucocorticoids, to bring about the establishment of immune complexes of lesser growths (in the case of larger ones).

The importance of attributing the activity to the process of carrying out the pulsterepathy allows for the recognition of small doses of glucocorticoids. Pulse therapy is most effective in young people with low triviality.

Likuvannya glucocorticoids do not depend on being successful, but it is reasoned:

The need for lowering the dose during development is accelerated, unaffected by those who are effective in such therapy for a particular patient;

Intolerance to glucocorticoids;

Resistance to glucocorticosteroid elimination (urge to die early).

In some cases (especially in the development of proliferative or membranous lupus nephritis), cytostatics are indicated: cyclophosphamide (a thousand intravenous bolus administered in a dose of 0.5-1 g / m2 for a stretch of not less than 6 m / s, 3 m / s for a skin length) given with prednisolone at a dose of 10-30 mg / add. Nadal you can turn to glucocorticosteroids until you get rid of glucocorticoids, if there is some resistance to them, you should know.

For the treatment of fewer severe, glucocorticoid-resistant symptoms of ailments, use azathioprine (1-4 mg / kg per dose) or methotrexate (15 mg / tizh) and cyclosporine (at doses less than 5 mg / kg per dose) ... doses of prednisolone (10-30 mg per dose).

Criteria for assessing the effectiveness of cytostatic storage:

Zmenshennya chi zniknennya key signs;

Testing steroid resistance;

Stike of decreased activity to the process;

Zapobіgannya progresuvannyu lupus-jade. Folded cytostatic therapy:

Leukopenia;

Anemia and thrombocytopenia;

Dyspeptic symptoms;

Infectious diseases accelerated.

With a decrease in the number of leukocytes less than 3.0x109 / l, the dose of the drug should change to 1 mg / kg mass. With a small leukopenia build-up, the drug should be skated and the dose of prednisolone will be increased by 50%.

Widely widened nabul extracorporeal methods of treatment - plasmapheresis and hemosorption. The stench allows you to see the organisms of the CVC, to increase the sensitivity of the cell receptors to glucocorticoids and to change the toxicity. Їх stasis in case of generalized vasculitis, severe organ damage (lupus nephritis, pneumonitis, cerebrovascular disease), as well as in case of swelling immune damages, as foul use glucocorticoids.

Call for extracorporeal methods of stasis at the same time with pulsterepsyu, for її ineffectiveness, independently. It means that the extracorporeal methods of cytopenic syndrome do not become stagnant.

We are sick because of the high titer of antiphospholipid antibodies in the blood, but without the clinical signs of antiphospholipid syndrome, we indicate small doses of acetylsalicylic acid (75 mg per dose). In case of confirmed antiphospholipid syndrome, which is superfluous with clinical signs, heparin is consumed with sodium and a small dose of acetylsalicylic acid.

For the treatment of musculoskeletal diseases (arthritis, arthralgia, mialgia) and serositis, it is possible to fix the specific dosage of the refinery.

Forecast

In the rest of the rock at the sound of the money effective methods The forecast has changed: after 10 years of diagnosis, the vision will become 80%, and after 20 years - 60%. In 10% of ailments, especially with severe nirok (death is insistent as a result of progression of chronic nirkova deficiency) or cerebrovascular disease, the prognosis is uncomfortable.

Prevention

Oskilki etiologiya VKV nevidoma, primary prophylaxis should not be carried out. Protect the group of children, before they bear it, the first for everything, the relatives of the ailments, and also the people who are suffering for the isolated children (disco-day vovchak). The stench of guilty of unique insolation, overcooling, not guilty, grab the chips, cut off the mud and the balneotherapy.

SYSTEM OF SCLERODERMIA

SSD is a systemic incapacitation of fatty tissue and other vessels, which are characterized by heat and enlargement of fibrous-sclerotic symptoms of the skin and internal organs. The cost of ailments is a fibrous transformation of the resulting tissue, which is a frame of internal organs, a storage element of a set of blood-bearing vessels. Non-observable development of dressing fibrosis from overworldly collagen solutions in case of deterioration of fibroblast function.

The expansion of the SSD growth in different geographical areas and ethnic groups, growth and in one region. Primary ailments range from 3.7 to 19.0 per 1 million population in Rik. The SSD is the most frequent restructuring of middle women (performance 5: 7.1) in 30-60 years of age.

Ethiology

The reason for the development of ailments is not home. Determine the value of viruses, some indications of indirect evidence of their role in the diagnosed SSD: in some tissues, virus-like inclusion was detected and the titer of anti-virus antibodies was adjusted. Established family-genetic instability before SSD, some of the relatives of the ailments see the symptoms of hypertrophy in viglyad hypergammaglobulinemia, Raynaud's syndrome, and in some cases SS.

Before nespriyatlivih faktorіv scho spriyayut manіfestatsії hvorobi that її zagostren, slіd vіdnesti factorization zovnіshnogo seredovischa (trivaly contact of polіvіnіlhloridom, kremnієvim pylome) zastosuvannya lіkarskih zasobіv (bleomіtsin, tryptophan), and takozh oholodzhennya, injury, torn down neyroendokrinnih funktsіy that vpliv profesіynih vіbratsії.

Pathogenesis

The basis of the pathogenesis is the destruction of the process of interaction of young cells (endothelial, smooth cells of the vertebral column, fibroblasts, T-and B-cell lymphocytes, monocytes, dangerous cells, eosinophilic cells) by one The result of all the overworked is the selection of a population of fibroblasts that are resistant to apoptosis and functional in an autonomous mode of maximum synthetic activity, which activates neofibrillogenesis and changes in the glucose of the main speech tissue. As a result, fibrous-sclerotic changes in the resulting tissue develop. One hour, one can see the dysregulation of the immune response to the organism on a vicious virus, so that it turns in the hyperproduct of antibodies to the wet tissues (autoantitil). It is possible to form immune complexes, to settle at the microcirculatory channel and to the internal organs, to bring about the development of immune firing. The variability of immune and autoimmune disorders in SJS is not as great as with VKV.

Fibrous-sclerotic changes from the resulting tissue, the defeat of the vessels and internal organs in response to the immune inflamed sensation, the versatility of the key signs of illness (Fig. 7-1).

Classification

In our country, I have adopted the classification of SSDs, as the nature of the breakdown, the stage of development of ailments and the clinical and morphological characteristics of the damaged organs and systems.

The nature of the leak:

Shvidko is progressing;

Chronic.

Stage:

Pochatkova;

generalized;

Thermal.

Small. 7-1. Pathogenesis of systemic scleroderma

Clinical-morphological characteristics of the striking:

Shkiri and peripheral vasculature - schilny nabryak, іnduration, hyperpigmentation, teleangiectasis, Raynaud's syndrome;

Support-arm apparatus - arthralgia, polyarthritis, pseudoarthritis, PM, calcification, osteoliasis;

Heart - myocardial dystrophy, cardiosclerosis, vada heart (most often - lack of valve);

Lungs - interstitial pneumonia, sclerosis, adhesive pleurisy;

Herbal systems - esophagitis, duodenitis, sprupodibny syndrome;

Nirok - spravzhnya scleroderma nirka, chronic diffuse glomerulonephritis, serebral glomerulonephritis;

Nervous system - polyneuritis, neuropsychiatric problems, vegetative destruction.

The variability of the grasping of the shkiri is assessed by palpation for a 4-point system:

0 - no damage;

1 - slightly impaired;

2 - about the loss;

3 - bend the fold (unwillingness to take the fold).

In the last few years, they began to see prescleroderma, diffuse scleroderma, intercourse (limitovan) scleroderma, which includes the syndrome CREST(As for the syndrome, it will be said below), і scleroderma without scleroderma (the whole option is even worse and becomes no more than 5% of all ailments with SJS).

For a chronic interruption, the most powerful STS, characterized by vasomotor breakdown, develops step by step, following the type of Raynaud's syndrome, and the enlargement of trophic problems with them, to serve as a single acquaintance of being caught by a stretch of rocky rock. It is necessary to come to a loss of school and periarticular tissues with the development of osteolysis and progressively progressive sclerotic changes in internal organs (stravovod, heart, leg).

A rapidly progressing current is characterized by the appearance of important fibrotic peripheral and visceral lesions in the first risk of ailments and uncomfortable lesions of nirok on the basis of a true scleroderma disease (the most frequent cause).

The progressive nature of the ailment, for the assessment of the evolution of the stage of the growth of the pathological process, there are three stages overcoming:

Stage I - cob manifestations - it is important to note the slopes of the serpent in case of pidgostroma, and vasospastic - in case of chronic course;

Stage II - generalization to the process - polysyndromism and poly-systemicity of the affected organisms and systems;

Stage III - thermal - the overhaul of severe sclerotic, dystrophic and necrotic processes (not infrequently - with varying functional impairments of one of several organs).

key picture

A clever picture of becoming ill and becoming polymorphic and polysyndromic, evoking generalizations of character. It is practically mute for the organ or systems, which could not have been received before the pathological process.

on the the first stage of the diagnostic joke It is possible to recognize information, on the basis of which it is possible to determine the diagnosis and variant of the ailment cob, the nature of the interruption to the process, the involvement in the pathological process of the organisms, the earlier carried out to accelerate this efficiency, as well as

Most of the sickness is to be repaired for the defeat of the shkiri, and then in the step-by-step it is necessary to repair the organs (typical form). In the last (atypical form) in the classroom pictures from the ear of the house, the deterioration of the internal organs in the case of the smallest childish symptoms, as well as the rapid diagnostics. In the world of getting overwhelmed, it is possible to say something about the nature of the overrun (gostre, pidgostre and chronic).

Scargias with ailments when they are diagnosed to a pathological process of internal organs show sub-active symptoms when they are severely affected (pleurisy, arthritis, Raynaud's syndrome, duodenitis, etc.). Water hour ailments can show scars, which are most characteristic of the SSO itself: the difficulty of coping and dying when it is difficult to get rid of the upper

parts of the stravohod. Vasospastic damage in Raynaud's syndrome is not surrounded by fingers, but widens on the hand and foot. Frequently, patients see a sense of the same in the area of ​​the lips, be it a part of the denunciation of the tongue. The smell of screeching on the dryness of the mucous membrane of the company and conjunctiva, as well as the unpleasantness of the poster (no tears). Defeat the shkiri of exposure to appear at the sight of the tightness of the shkiri that company (it is important to see the mouth). As a rule, the temperature is not adjusted. Skhudnennya (in one meaning), by all means, beloved in case of progress and generalization of ill-health.

For the first stage (with a trivial overload of ailments), it is possible to develop a new diagnosis. Viconation on the very cob is very important, because the symptoms of SJS are a lot in which to think of the disease in the group of DZST (VKV, RA, DM), and in case of mono-oligosyndromosity, it is a disease of the disease, which is characterized by one .).

Ha to another stage of a diagnostic joke rymuyut the data, what about the destruction of organs and systems and their functional shortcomings. With the open-minded clinical picture of ailments, the fight against children means a large number of ailments. It will appear at the last development of the embryo, induration, that atrophy with the important localization in the eyes and hands. There can also be trophical snakes of children at the sight of depigmentation, an uncared-for child of the judgment and teleangiectasis. The cut of the mucous membranes appears in the dryness. On the shkіrі there can be a winikati virazki and a gnіynichkovy visip; hairy vipadaє, nіgti deform. At the end of the stage, the ailments of the shkira are denounced as shitty, and it is uncomfortable to take them into the fold. The person is amic, mask-like. The form of the company is characteristic: lips are thin, taken into folds, but it is not distributed, the structure is gradually drawn into the mouth until the mouth is wide (symptom of a “pouch”).

Vasospastic changes in Raynaud's syndrome in the eyes of the skin surface appear in the area of ​​the face, lips, hands and feet.

Determination of the slopes can be seen in their defigrations for the development of periarticular tissues, as well as for secondary sclerodermic polyarthritis for the development of exudative-proliferative abnormal fibrosis-inductive. Characteristic is the development of the scleroderma hand: shortening of the fingers due to osteolysis of the nigth phalanges, the vitrification of the nipple bones, deformation of the nigths and mild original contractures. Taku a brush to break from a bird's paw (sclerodactylia).

The dissection of the mucosa, morphologically, is fibrous interstitial myositis, or myositis with dystrophic and necrotic changes, manifesting itself in myasthenic syndrome, atrophy, and altered muscle mass and deterioration. It is more possible to approve the sickly gullet (calcification) at the myaz. Especially often, calcium salts are added to the soft tissues of the fingers.

Urazhennya shKT (esophagitis, duodenitis, a syndrome of damaged skin or constipation) is mainly manifested on the first and third stage of the diagnostic joke.

Determination of the organs of energy appears in the eyes of pneumonia, which is chronically opposed to the state of health, mlyavo. Physical data in the wretched land, in the swivel in the fall, they deprive the emphysema of the legend. Significantly great information is given by X-ray dosage, which is necessary for the sutta to help with the development of bilateral basal pneumosclerosis, characteristic of SJS.

In case of severe pneumosclerosis and trivial one, Legenevian hypertension develops, which leads to the hypertrophy of the right slunk, and then - to its deficiency. Legeneva hypertension manifest cyanosis, accent II tone in another mid-ribbed malignancy from the sternum, a backwardness, a sharp decrease in tolerance to physical resistance and changes in the strength of pulsating in the heart

Determination of the heart of the problem is the main point in the middle of the visceral symptoms of SJS, both by frequency, and by pouring on the result of ailments. For SS, the so-called primary cardiosclerosis is characteristic, not tying because of the necrotic and fiery changes of the myocardium. In addition, it means an increase in the heart (inodi - meaning), as well as a deterioration of the heart rhythm in the view of the extrasystole of MA. Striking the endocardium to develop to the development of the heart, it is practical to start it - to the mental deficiency. The presence of cardiosclerosis in a number of diseases can be increased by the development of heart failure with characteristic signs. Pericarditis with SS is usually rare and most often dry.

Separation of other vessels - scleroderma angiopathy - manifestation of vasomotor impairments (Raynaud's syndrome) and characterized by paroxysmal vasospasm with a characteristic after-time changes in the formation of a small number of fingers (damage to the heart, cyanosis) In severe cases, Raynaud's syndrome can lead to hemorrhages, tissue necrosis of the fingers and teleangiectasis.

The smacking of nirok in SS (in 80% of ailments) is accompanied by pathological symptoms of sudin, ale with development of fibrosis. A most serious symptom is a scleroderma nircovy crisis, but you should develop in the first five years of ailments in ailments with a diffuse form of SSD and a man-festering malignant AG (AT more than 170/130 mm Hg) in 90% of those with nonspecific signs.

In the basis of the nervous system damage, there are dystrophic and fibrous changes, presented by symptoms of polyneuritis with impaired reflexes and sensitivity.

In such a rank, for another stage, the polyorganism of the battle against the overwhelming battles of the shkiri and її derivatives appears. The step of change is even more pronounced - from subclinical to significant turns. Possibility of establishing the diagnosis of SSD in case of a badly affected school

more visoka, less near visceral porosity. In the last resort, if the first plan is to strike at one organ (nirka, heart), to change the mind for the establishment of diagnostic pardons.

You can:

Visualize the steps of the process;

specify the turnover of internal organs;

Carry out a differential diagnosis of the other illnesses from the group of chronic DZST.

At the designated level of activity, the most significant are nonspecific acute phase indicators, such as:

Disproteinemia with adjustments to the concentration of a 2 - і -globulin;

submission to the SRB deputy;

Increased concentration of fibrinogen;

Pidvischennya SHOE.

One can judge about the manifestation and turnover of immune disorders by the values ​​of the Russian Federation (found in 40-50% of patients), antinuclear antibodies (in 95%) and LE-clitin (in 2-7% of patients). On the basis of VKV, all indicators with SKD show significantly lower titers and higher.

The most important diagnostic value is given to the so-called sclerodermic antibodies.

Antibody Scl-70 is most often found in diffuse forms of SSD (40%). The presence of HLA-DR3 / DRw52 in a person with a nose is a prognostically unpleasant factor in ailments with Raynaud's syndrome, and the development of lung fibrosis in patients with SS in 17 cases.

Antityla to centromera (chromosome element) occurs in 20-30% of ailments (there are more signs of CREST syndrome).

Antibody to RNA polymerase I and III highly specific for SSD. The stench of the presence is important in ailments with a diffuse form and associated with lesions and an unpleasant prognosis.

When defeated, it turns out to be in this world of protein in the midst of the smallest changes of a slaughter siege (microhematuria, cylindrical structure). In case of normal scleroderma nyrci (development of necrosis of nirk tissue as a result of deterioration of nirk nyrkas), the lack of creatine in the blood may develop.

In case of SS, it means dissociation and occurrences in punctual biopsies with morphological changes of nirk tissue and sudin and occasionally with other clinical (including laboratory) diagnostics of damage. If arterial hypertension develops as a result of the development of arterial hypertension, then it means changes in the bottom (arterial hypertension and enlarged veins).

If the heart is damaged on the EKG, nonspecific changes in the end portion of the slurry complex start (decrease in the amplitude and inversion of the tooth) T), and inodі - the deterioration of the internal drainage capacity. Radiographically visualize the improvement of the heart. X-ray of pre-help

to develop calcification of myazia and soft tissues of the fingers of the hand, as well as differentiation of serpentine lobes in SSD due to damage in RA (in SSA in the daytime erosion of the loaf surfaces). In 60-70% of cases on roentgenograms, it is obvious that the intestinal tract is affected (especially in the intestinal tract). The worms of the stratohode are represented by diffuse enlargements in the second, from the bellies in the lower third, weakened peristalsis and a decrease in the rigidity of the walls.

In case of biopsies, synovial membranes, and mucous membranes, they appear characteristic of SSD, fibrous changes, as well as damage to sudin. The date of morphological dosage does not give a general meaning to the established diagnosis.

Diagnostics

The diagnosis of ailments is based on the findings of the great and small diagnostic criteria.

To greater criteria, tolerate proximal scleroderma - symmetrically sweating, hardening and indigestion of the number of fingers and shkiri, rosette proximal to the lower papal-phalangeal and metatarsus The serpents can conceive of denunciations, shiyu that tulub (chest wall that lives).

Small criteria:

Sclerodactylia - pererakhovanі vische shkіrnі zmіni, intermingled zaluchennyam in pathological process of fingers;

Scars on the fingers of the fingers;

Bilateral basal legenevium fibrosis.

A sick SSD has a big criterion (great), or, take, two small criteria. Sensitivity - 97%, specificity - 98%.

The most common type for SJS after calcification, Raynaud's syndrome, esophagitis, sclerodactylitis and teleangiectasis (syndrome CREST- Behind the first letters of the English naymenuvan of symptoms).

Diagnosis of SS at the early stages is based on the revealed three primary signs (when they are most diagnosed early): Raynaud's syndrome, lumpy syndrome (often polyarthralgia), and shkіri shkіri. However, at the early stage, one of the visceral localization processes appears.

Significant difficulties in diagnostics of SJS are associated with the appearance of a characteristic shkirny syndrome in ailments because of abnormal polysyndromic lesions of internal organs (the so-called SJS without scleroderma). In cich vipadkah, I will definitely need to help you with a radiological dosage, so that it allows you to see impaired motility in the traumatic duct of that extension, as well as dilatation of twelve-fingered intestines.

Differential diagnosis

SSD followed the differentiation from a whole series of illnesses and, the first for everything, from the DZST, and also from ailments, the key picture of which is even more similar to such a defeat of a certain body with SSD (for the mind of one

minuvannya). For example, in case of scleroderma urazhenny heart to carry out differential diagnosis of atherosclerotic cardiosclerosis, rheumatic heart disease and nonspecific myocarditis; in case of legene urazhenny - with chronic pneumonia, tuberculosis and professional illnesses (pneumoconiosis); when urazhennі stravohodu slіd viklyuchiti th cancer.

The basis for differential diagnostics є detection of typical signs for SSD.

Reconsideration of adult children with Reynaud's syndrome and slight variations in laboratory data in case of SS on the type of small children with VKV, in order to experience a greater activity of pre-existing pathological process.

On the basis of VKV, with SSD, the defeat of internal organs cannot be met with the development of immune damage (ANF, RF and antibodies to DNA appear at a lower titer, the frequency of such a number of LE-clitin is also insignificant).

Suglobovy syndrome with SS on the form of the RA is associated with meat contractures, calcium deposition in soft tissues and tissues, fibrous ankylosis and osteolysis of the pineal phalanges. Destructive changes in the cyst tissue in case of SSD in the daytime, overtaking the damage of the periarticular tissue

On the day of the IXC, heart damage with SJS does not lead to angina pain. On the EKG there are signs of the transferred IM. On the appearance of rheumatic lesions of the heart, stenosis does not develop with SSD of nicoli (mitral, aortic throat); hesitate to the point where the mental deficiency is turned around.

Dominating the problem of any system, or the organ with SJS, is likely to be affected by the shinny and nutritious changes and Raynaud's syndrome. Monosyndromism is characteristic of the clinical picture of those with ailments (chronic pneumonia, atherosclerotic cardiosclerosis, bowel disease, viral ailment).

In SSD, there is a dominance of minor changes and Raynaud's syndrome, and in DM, the first plan is to start the fight against mucous membranes in a single paraorbital swelling of the tuberous collar ("symptom of the eyepieces").

Glucocorticoids with SJS do not give such a fiery positive effect as with VKV.

In a number of vipadkiv, if SSD manifestus with a globular, shkinny and asthenovegetative syndrome, the trivial dynamic caution is not allowed to make the correct diagnosis.

The formulation of open-ended key diagnostics can be found in the headings, guided by the working classifications. Diagnosis is guilty of displaying:

The nature of the leak;

Stage;

Clinical and morphological characteristics of the affected organs and systems, organisms from the stages of functional deficiencies (for example

world, in case of pneumosclerosis - the stage of legacy deficiency, in case of severe nirok - the stage of normal deficiency and in.).

Likuvannya

Lіkuvannya SSD maє buti complex and vrahovuvati navnі aspects:

Inflow to Sudinni accelerated and Pershu Chergu - to Raynaud's syndrome;

Injection for the development of fibrous changes;

Immunosuppression and anti-firing;

Injection on local symptoms of illness.

Slid unique in the flow of cold, curvature, muscle injections, stress situations and drug intake, as well as peripheral vascular spasm (β-adrenergic blockers without vasodilatation).

Medication for the treatment of Raynaud's syndrome is a sign of blockers in the main calcium channels - amlodipin (5-20 mg per dob), prolonged nifedipin (30-90 mg per dobu), felodipin (5-10 mg per dobu) (240-480 mg per dobu) or diltiazem (120-360 mg per dobu).

A good effect is taken internally with pentoxifyline (400 mg 3 times per day). It also priznachayut antiagreganti - dipyridamole (300-400 mg per dobu) or ticlopidin (500 mg per dobu).

In critical situations (Legeneva hypertension, gangrene, nirkovy crisis) for 6-24 years for 2-5 dib, intravenously inject synthetic prostaglandins: alprostadil (0.1-0.4 mcg / kg per quiline) or iloprost (0.5 -2 ng / kg per quiline).

The drug, which is responsible for internal linkages in the collagen molecule and the hemorrhage of the collagen solution, is penicylamine. Yogo priznachayut in case of advanced leaks, rapidly growing, indulgent changes in children and symptoms of progressive generalized fibrosis in a day at a dose of 250-500 mg per dose. Earlier, the recommended high doses (750-1000 mg per additional dose) did not increase the efficiency of treatment, but the frequency of the treatment of secondary events was significantly increased. When using penicylamine, it is necessary to follow the laboratory indicators of the slash, a mark for 6-12 m / s from the moment the ear is picked up, proteinuria may develop. When її її grows up to 0.2 g per dob, the drug should be skated. In case of swelling urazhennya, enzyme therapy is recommended. It means that the introduction of hyaluronidase in the vicinity of the disease or electrophoresis with the drug is considered.

Protisapalny and cytotoxic drugs are stagnant at the early (ignited) stage of SSD and with a rapidly progressing interruption of the disease.

Glucocorticoids in small doses (15-20 mg / dob) stagnate in case of progressive diffuse uterine symptoms and obvious clinical signs of fiery activity (myositis, alveolitis, serositis, refractory).

arthritis and tendosynovitis). Reception of large doses of non-recommendations (risk of development of scleroderma nirkovy crisis).

When prescribed in a dose of 2 mg / kg per day for 12 months, cyclophosphamide changes the pressure of the shkiri tilka in ailments to the diffuse form of SSD.

Methotrexate should be used in case of SSD from RA or PM.

In case of scleroderma nirkovy crisis, for the reduction of convulsive spasms and the prevention of the development of scleroderma nirka vikoristovyt ingibitori ACE (captopril, 100-150 mg per dob, enalapril, 10-40 mg per dobu) under the control of arteri.

When urazhennі stravohodu with the method of prevention of dysphagia, it is recommended to often take a shot and take it out of the age of 18 years. Treatment of dysphagia in the transmission of prokinetic agents (metoclopramide at a dose of 10 mg 3-4 times per dose). In case of reflux esophagitis, omeprazole should be used (vseredin 20 mg per dose).

Injection on local symptoms of ailments transfer to aplikatsiyu 25-50% range of dimethyl sulfoxide. During the period of activity of the pathological process, exercise therapy and massage can be recommended.

Forecast

In case of SSD, the prognosis starts with the option of overcoming that stage of development. Apparently, more than an hour after the rise to the Kremlin stage, the first signs of ailment (Raynaud's Syndrome) have a favorable prognosis. The five-fold vision rises from 34 to 73%, while the average stance is 68%. The risk of death in SJS in 4.7 is developmental, but not in the population.

Predictor of a harsh prognosis:

diffuse form of ailment;

On a cob of ailment older than 47 years;

How many to become;

Fibrosis is a legend, a legendary hypertension, arrhythmias, a fight against nirok in the first three diseases of ailments;

Anemia, temporal SHOE, proteinuria on the cob of ailments.

Prevention

Before a group of patients, they are susceptible to vasospastic reactions, polyarthralgia, and also relatives of ailments, who are hungry for the development of diffuse illnesses of other tissue. The stench is not guilty of being injected with provocative factors (coldness, vibration, trauma, bad words, infectious agents, etc.). The sick person on the SSD should be placed at the dispensary obl_k. Systematic implementation of the treatment (zokrem, correct selection of the appropriate therapy) - the best prophylaxis zagostren

DERMATOMIOSIT (POLIMIOSIT)

DM is a systemic fever of skeletal, smooth muscles and shkiri. Ridge mean the loss to the pathological process of the internal organs. For the duration of the day, the school is vicious, the term polymyosit PM.

The main symptom of a malady is a weakness of myositis due to progressing severe necrotic myositis with major lesions of the proximal symptoms. In the world of progresuvannya m'yazovannya tissue atrophy and replace fibrous. Similar processes occur in myocardium. Dystrophic changes develop in parenchymal organs. Before the pathological process, judges of muses, internal organs and shkiri are also affected.

DM (PM) is a rare affliction. The incidence rate in the population ranges from 2 to 10 per million inhabitants per Rik. Ailments are sick up to people of adulthood (40-60 years), often - people, not women (2: 1).

Ethiology

I see two forms of DM (PM) - idiopathic and secondary (chubby). The ethiology of the idiopathic DM is unclear, alas, it is seen as a factor, which is taken from the Manfestats, but it is also necessary for the aggravation of the disease:

Insolation;

Overcooling;

Infectious disease (GRZ, flu, angina and in.);

Hormonal overbudding (climax, vaginist, canopy);

Emotional stress;

physical trauma, surgical involvement;

Sensitization drugs(chlorpromazine, insulin preparations, antibiotics, penicylamine);

Vaccination;

Contact with epoxy resins, photocells;

Physiotherapy procedures.

Ymovirno, there is a significant slump-genetic strength: ailments have antigens B-8 / DR3, B14 and B40 of the HLA system. The price is clearly not linked to the sickness themselves, but to the singular immune disorders and in the first place from the hyperproducts of myosinspecific autoantitils.

Chubby (secondary) DM to become 25% of all types of illnesses and develop among ailments who suffer from ill-gotten fluff. Most often DM is diagnosed with cancer of the lungs, intestines, anterior malignancies, ovary, as well as with hematological malignancies. The winery of the DM in particular for 60 years may be ordered for the first time.

Pathogenesis

Given the infusion of a virus and genetic schlichnosti or chubby antigens, the deterioration (dysregulation) of the immune

imbalances in B- and T-lymphocyte systems: in the body, antibodies are violated to skeletal mucus and sensitization of T-lymphocytes develops before them. The reaction "antigen-antibodies" and the cytotoxic effect of sensitized T-lymphocytes to the mucous membranes are approved and deposited in the microcirculatory channels of the immune complex organs. Х elimination to produce lysosomal enzymes and the development of immune firing in the tissues and internal organs. When ignited, new antigens are evolved, they are taken to a false setting of immune complexes, until the chronization of illness and disease before the pathological process of earlier healthy mutilations. The main lines of the pathogenesis of DM are presented in Fig. 7-2.

Small. 7-2. Pathogenesis of dermatomyositis

key picture

A clever picture of becoming ill is systematic and polysyndromic.

Main syndromes:

Myazovy (myositis, myazova atrophy, calcification);

Shkirny (eritema, shkiri embryo, dermatitis, pigmentation and depigmentation, teleangiectasis, hyperkeratosis, cropping);

Suglobovy (arthralgia, striking of periarticular tissues, rare - reference arthritis);

Visceral (myocarditis, cardiosclerosis, pneumonitis, aspirating pneumonia, pneumofibrosis, intestinal bleeding, myoglo-

bulinuric nirka with the development of hostile nirkova deficiency, polyneuropathy). I see such a period when I get sick:

Period I (cob) - three days from a few days up to 1 month and more, manifesting more and more, and (abo) shkirnyy zmіnami;

II period (manifestny) - the picture of the ailment is flared;

III period (thermal) - representations of dystrophic changes in internal organs and signs of a widespread functional deficiency (rapid development of rapid development).

There are three forms to break the ailments:

Gostru form, if the growth of generalized growth of skeletal muscles is rapidly increasing, it is possible to produce the sick person to the extent that it becomes more unruly. Progression of the mucous membrane of the pharyngeal ring and stravohode (dysphagia, dysarthria). The battle of internal organs (especially the heart) develops quickly with a lethal end in 2-6 months from the ear of ailment;

Pidgostru form with more, we will act on the build-up of symptoms. A bitter battle of music and viscerality will come through 1-2 rockets;

Chronic form with trivial cyclic interruption. Overcome the processes of atrophy and sclerosis. You can locally fight myaz_v.

on the the first stage of the diagnostic joke to recognize the views about the nature of the ear of a sickness - hospitality (adjusting the temperature up to 38-39 ° C, the school of the problem and pain in the muscles) or actions (in spite of the weakness, inappropriate thinking, ).

Some of the most characteristic scars of the muffs: ailments mean weakness, they cannot stand on their own, but to get up, it’s very important to come down to the ground, it’s not too big for the sounds. M'yazovaya weakness and soreness are localized symmetrically in the proximal parts of the teeth, back and shoulders.

When the throat muses are struck, it’s ailing to choke when it’s blown, it’s a little wobbly. The nasal voice is similar to the hoarseness of the larynx muzzles.

In case of severe ailments, it means that there is a change in storage in the scenes that are sick up to the day of the sun (decollete zone, disguises, brushes), as well as on the inviting surfaces of the stegon and homosexuals. Characteristic is the diagnosis of the paraorbital nodule of the buzkovy kolor ("symptom of the eyepieces"). In case of severe mucous membranes, the ailment scars on dryness, pain in the eyes and the appearance of tears ("dry" syndrome).

The development of a pathological process of other organs to develop symptoms that lead to myocarditis, cardiosclerosis, pneumonitis, glomerulonephritis, polyneuritis, arthritis, etc.

On the one hand, about the test, one can judge about the correct diet, and, bit by bit, about the nature of the change: the test of aminochinolin drugs in evidence of a chronic leak, the stagnation of prenatal and cytostatics - about the guest.

on the to another stage of a diagnostic joke with open-ended clinical pictures of ailments, in front, by and large, there is a symmetrical fight against the symptoms: strong, testy on the dot, the smell of sickness and pain on palpation. When the mimic musculature is striking, the deyak is thought to be masked. Nadal vynikak atrophy of muscular tissues, especially swirls from the side of the shoulder girdle. It is also possible to see the battle between diaphragms and diaphragms. With palpation of muscle tissue, it is possible to detect localized damage - calcification, which can grow in the middle fat cells. Calcification often develops in young people with an increase in muscular tissue damage during the transition from a health current to a chronic disease. The decrease in weight by 10-20 kg is not often spared.

The problem of shkiri is not a sign of the DM, but when it is shown on the top of the critical parts of the head, it means a bulge, an erythema (over the slopes - over the slopes, in the colony zones in the dark, some Erіthema leads to a great style, cyanosis, to supervise the sverbіnnya and huskies. Typical "symptom of the eyepieces" is an erythema near the eyes. It is not uncommon for the peeling, peeling and development of shkiri dolon ("the hand of a mechanic or a repairman");

To achieve frequent restructuring of bending Raynaud's syndrome.

Signs of visceral lesions with DM, yak i, yak i with STD, even yaskravi, on the VKV view. It is possible to refer to the dissociation between the turn of the pathomorphological changes of the organs and the clinical manifestation. Disorder of the heart (myocarditis, cardiosclerosis) is represented by such nonspecific signs, such as an increase in the size of the heart, deafness of tones, tachycardia, and a disturbance in the rhythm of the extrasystole. Swelling of the myocardium can lead to symptoms of heart failure.

Depressing the lungs yak pneumonitis supravodzhutsya in the region miserable symptoms. Fibrosis, scho to develop, appears behind the signs of emfizemia, a legend and a dysfunctional lack. Aspiracy pneumonia is characterized by all typical symptoms.

Dysphagia is characteristic of the reduction of the intestinal tract: dysphagia: it is difficult to see solidity through nis ridkoi їzhі. Pathological changes in the intestinal tract can lead to shlunkovo-intestinal bleeding. In some cases, in addition to the improvement of the liver, later - hepatological syndrome from the development of lymphatic universities.

Neurological impairment is represented by signs of sensitivity: hyperesthesia of the peripheral abnormal nature, hyperalgesia, paresthesia and reflexology.

on the the third stage of the diagnostic joke Often I will help you to find methods of follow-up, which will allow you to assess the state of the ignition process and the breadth of the response.

The process can be judged by nonspecific acute phase indicators (increase in SHOE, advancing fibrinogen and CRP,

hyper-a 2 -globulinemia) and sign of immune changes (low titer of the Russian Federation, improvement instead of γ-globules, antibodies to nucleoproteins of nuclear antigens, antibodies to Mi2, Jol, SRP, and a pathway for different Ig

In case of chronic, low-grade sickness, acute phase indicators may be visible (SHOE is not very normal).

The breadth of the lesion is characterized by low biochemistry. The creatine / creatine index is increased, which is associated with the presence of creatine in the serum with a decrease in creatine. With a significant lesion, there can be a diagnosis of myoglobinuria. Increased activity of transaminases is not typical for the improvement of skeletal muscles. In some people with ailments, hepatitis is susceptible to myopathic syndrome.

When immunologic predictions are detected, myositis specific antibodies are detected. Antibodies to aminoacylsynthetases of transport RNA (antisynthetase antibodies) and antibodies to histidyl-tRNAsynthetase (Jo1) are transferred to them. Antibody Jo1 is found in half of the ailments on DM (PM), and only anti-synthetase antibodies are rare (5%). The products of antisynthetase antibodies are associated with the development of the so-called antisynthetase syndrome, which is characterized by cob, fever, symmetric arthritis, interstitial lesions, Raynaud's syndrome and hand wounds.

For DM of chubby walking, in choloviks, a prostate-specific antigen is characteristic, in females - CA-125 (ovine fluff antigen). In addition, in case of localization of chickens, specific antigens can be detected.

Often I will help in diagnostics of the mucous membrane by means of electromyography, so that it allows the normal electric activity of the mucous to develop in the country with a high degree of relaxation and low amplitude - at a very high speed.

In case of biopsy of the muscular tissue, it means a picture of important myositis with the second transverse darkness of the muscular fibers, fragmentation, granular and wax-like degeneration, and also in the presence of necrosis, lymphocytic plasma To carry out myazovu biopsies to confirm the diagnosis of the DM to look for the manifestation of the characteristic clinical, laboratory and instrumental signs of discomfort. The most informative biopsy of the meat, obtained before the pathological process, or without the atrophied atrophy.

The other methods of pre-examination (EKG, radiological and endoscopic) are necessary for:

Assessments of internal organs;

Poohlini pohlini when you grow up on DM pohlinnogo pokhlinnaya.

Diagnostics

For the diagnosis of DM, the following diagnostic criteria are followed.

Training shkiri:

Heliotropic visip (purple-chervony visipania on beads);

Signs of Gottron (purple-chervona atrophic erithema, peeling off, or by flicking on the original surface of the brushes above the slopes);

Erythema on the original surface of the pictures over the littoral and collinear slopes.

Proximal muscle weakness (upper and lower ends and tulub).

Increasing the activity of CPK chi aldolazi in the blood.

Pain in the muscles with palpation of the muscle.

Myogenic changes in electromyography (short polyphase potentials of motor units with spontaneous fibrillation potentials).

Detection of antibodies Jo1 (antibodies to histidyl-tRNA synthetase).

Non-destructive arthritis of chi arthralgia.

Signs of systemic firing (temperature adjustment at 37 ° C, increase in CRP concentration or SHOE at 20 mm / year).

Morphological changes that lead to firing myositis (fired infiltrates in cystaceous meats with degeneration or necrosis of the muscular fibers, active phagocytosis, or signs of active regeneration).

In case of detection of one type of defense, the diagnosis of DM is reliable (sensitivity - 94.1%, specificity - 90.3%).

The presence of accepting choter is indicative of a diagnosis of PM (sensitivity - 98.9%, specificity - 95.2%).

Differential diagnosis

Unimportant to the high sensitivity and specificity of the criteria, the diagnosis of the DM (PM) becomes very difficult, especially at the debut of the disease.

DM (PM) followed by differentiation from infectious and neurological illnesses, SSD, VKV and RA. The basis of differential diagnostics is based on the following changes:

The incidence of loamy syndrome in RA, the appearance of radiological pre-diagnosed erosions of the loamy surfaces of the cysts, the appearance of changes typical for DM.

At the viewpoint of the VKV, with the DM, the decrease in the minority rapidly changes and the decrease is significantly higher. In the clinical picture of DM, there is a lot of mucus, and laboratory indicators (especially immunological) change significantly in a smaller world.

On the view of the SSD, there is a slight change in character with DM: there are no typical changes in the hands, and we have muzzle syndrome (including the weakness of the muscles). Protect differential diagnostics of SSD and DM naivazhcha. In folding vipadki, it is necessary to use electrophysiological and morphological methods of preliminaries.

In case of a hospital leak, the DM needs to switch on infectious disease (septic tank, beshikhov fired up and in), as well as possible with a dynamic guard for ailments.

In case of dominant adynamy and impaired reflexes of the patient, it is necessary to carry out differential diagnostics for neurological disabilities, as well as when a patient is spoken to by a physician or a neuropathologist.

The formula for the open key diagnostics of the DM can display:

period of current;

Leakage form;

Clinical and morphological characteristics of the system of organs and organs due to the significance of the provincial syndromes and the lack of functional deficiencies of organs (systems).

Likuvannya

Mainly zavdannya - suppression of the activity of immune reactions and the ignition process, as well as normalization of the functions of the environment, the most effective organs and systems. An early ear of licking (extending the first 3 months at the time of the onset of symptoms) is associated with a more friendly prognosis, lower than that.

The best effect may be glucocorticoid: with DM, prenizolone should be used (1-2 mg / kg per dose). With a hold of the first tendencies, I will add a dose of three hours, and then take the whole one-time vrance, some of the sickness will grow more and more, lower with VKV chi SSD (in the middle one - in 1-3 months). For the duration of the positive dynamics for 4 days, the dose of glucocorticoids was increased. In order to reach the effect (normalization of the muscular force or the activity of CPK), the dose of prenisolone should be reduced even more frequently to an acceptable level, and by 1/4 of the total. Reducing the dose is necessary to carry out with suvorim clinical and laboratory control.

Pulse therapy is rarely effective. Її priznachayut in case of rapid progressing dysphagia (risik of aspiracy pneumonia) and the development of systemic lesions (myocarditis, alveolitis).

It’s also not effective to use prenizolone, or it’s unwise to indicate that there is a history of intolerance, and the development of an accelerated development, followed by the use of cytostatic drugs.

In the last hour, it was recommended to use early methotrexate, allowing the patient to transfer the ailment to an acceptable dose of prednisolone. Methotrexate is used internally, pediatricly or internally at a dose of 7.5-25 mg per day. Intra-intravenous administration of the drug is recommended in case of lack of efficacy or lack of tolerance when receiving internally. As a reminder, the appearance of the effect as a prenizolone depends on the possibility of using the plump ANF, so before that, in order to initiate cytostatic drugs, it is necessary to carry out the expansion of the malignant problem for the prevention.

We are sick with forms resistant to predisolone, priznachayut with internal cyclosporine at 2.5-5.0 mg / kg per dose.

Azatioprine is used for methotrexate effectiveness. The maximum effect develops lower (in the middle one - in 6-9 m / s). Assume that the drug is taken internally at 100-200 mg per dose.

Cyclophosphamide - as a vibor for interstitial fibrosis of legenev (2 mg / kg per dose).

Aminochloroquin preparations (chloroquin, hydroxychloroquin) stagnate in such situations:

In case of chronic interruption of ailments without a sign of the activity of the process (control of shkіrnyh patches);

At a reduced dose of prednisolone, or cytostatics, the reduction of the risk of mossy zagostrennya.

Plasmapheresis is a sign of ailment due to severe, resistant to other methods of treatment of DM (PM) in patients with glucocorticoids and methotrexate or cytostatic drugs.

In the rest of the rock for lіkuvannya all the more often zasosovat іngіbіtori FNP-α. Promising direct dressing treatment from rituximab use. The maximum effect develops after 12 tendencies during the first period of time, together with the decrease in the amount of CD20 + lymphocytes in peripheral blood.

Forecast

At the same time, the prognosis has changed significantly: the five-point vision becomes 90%. At the time of the illness of a chronic person, the patronage of a sick person can be seen.

The prognosis for secondary (chubby) DM is based on the efficiency of the operative involvement: for a successful operation, all signs of ailments can be known. Officials, who can burn the forecast of ailments: lіtnіy vіk, diagnosis, improper treatment of ailments on the cob, severe overload of myositis (fever, dysphagia, urazhennya legen, heart and CT), antisynthetase syndrome. With a chubby DM, the p'yatirichne vision becomes less than 50%.

Prevention

Increased burnout (secondary prevention) is within the reach of ongoing treatment, sanitation in diagnostics and diagnostics of organisms. The relatives of the sick can undergo primary prophylaxis (prevention of re-entanglement, insolation, overcooling).

Systemic impairment with the resulting fabric

1. Newsletter

Systemic chervonia vovchak, systemic scleroderma, dermatomyositis-polymyositis can be attributed to systemic diseases of the associated tissue (SZST) - a group of nosologically independent ailments, but there is little similarity in etiology, pathogenesis, and key diseases. The treatment is carried out with certain preparations.

A major moment in the ethology of all SZSTs is the latent infection with small viruses. Due to the deterioration of the tissue tropism of viruses, the genetic schism of the ailing, it is possible to develop the development of the disease of the whole group.

The triggers are triggered by the mechanisms involved in the pathogenetic processes of the SZST nonspecific. Most often, overcooling, physical activity (variation), vaccination, intercurrent virus infection.

The winery under the inflow of the trigger factor, the splash of immunoreactivity in the organism of a sickly sick person appears not to die on its own. As a result of antigenic mimicry of virus infections, the clitin is formed more viciously than the ignition process, which self-develops, leading to the degradation of all systems and specialized tissue structures in the organism of tissue that is sick to fatty tissue. The name is old, the name of the group of ailments is colagenosi.

All SZSTs are characterized by the defeat of the epithelial structures - shkiri, mucous, epithelial vases of the zonal secretion. In addition, one of the typical clinical manifestations of a group of ailments is dry Sjogren's syndrome.

Obviously, this world is filled with mucous membranes, serous and synovial membranes, which manifest themselves as mialgia, arthralgia, polyserositis.

Systemic lesions of organs and tissues in case of FDST with the formation of obov'yazkovo formulation for all ailments of the whole group of secondary immunocomplex vasculitis of the middle and other vasculature, including microscopic ones, to take part in the microscopy.

A typical manifestation of an immune complex vasculitis is an angiospastic Raynaud's syndrome, an obovascular component of a clinical picture of all illnesses from a group, which can be looked at.

At the most significant link between all the SZSTs, there are clinical symptoms with overconfident signs of the same disease from a group of people, for example, systemic worm disease, systemic scleroderma, dermatomyosis. In such cases, one can read about the disease of diffusion from the resulting tissue - Sharpe's syndrome.

... Systemic chervona vovchanka

good luck with vovchak polyositis

Viznachennya

Systemic chervonia vovchak (VKV) - diffusely engulfed tissue from the earliest tissues to the structural elements of tissues, components of cell nuclei, circulating in the blood of immune complexes of conjugated with an active complement of healthy immune cells

Ethiology

It is more likely to develop in individuals with HLA DR2 and DR3, in families with a deficiency of additional complement components. The ethiological role can be played by the infection of RNA-retroviruses from the group of "povilnye". Launch the pathogenetic mechanism of VKV can be intensive sleepy insolation, lykarski, toxic, nonspecific infectious diseases, vagina. Before falling ill, women were 15-35 years old.

Pathogenesis

Genetic defect and / or species change by "common" retroviruses of the genetic basis of the immune system and zooming in dysregulation of the immune response on the basis of certain events. The winery is overcrowded in immunoreactivity due to displacements in the range of antigens of normal tissue and internal structures.

A wide range of autoantitils is formed, which may cause aggressiveness to wet tissues. Among these are autoantitils against native DNA, polypeptides of short nuclear RNA (anti-Sm), polypeptides of ribonucleoprotein (anti-RNP), RNA-polymerase (anti-Ro), protein at the storage of RNA (anti-La), cardiol antiol histones, neurons, blood cells - lymphocytes, erythrocytes, thrombocytes and in.

In the blood there are immune complexes, building up with complement and activation. In front of the IgM complex with native DNA. Conjugate immune complexes with an active complement are fixed on the stage of the brain, on the tissues of the internal organs. The system of microphages accumulates in a large number of neutrophils, as in the process of destruction of immune complexes, a large number of proteases are observed, and atomic muslin is seen in its cytoplasm. At the same time with proteases, the active complement of the substance is used to stimulate tissue, judge. Immediately through the C3 component of the complement, the processes of fibrinogenesis and further synthesis of collagen are activated.

An immune attack on lymphocytes with autoantitiles, which reacts with a DNA-histone complex and an active complement, ends with lymphocytes ruining, and the nuclei phagocytes with neutrophils. Neutrophils, which take away the nuclear material of lymphocytes from the cytoplasm, are called LE-cells. Tseclassic marker of the systemic chervony vovchak.

key picture

Klinichny overbig VKV can be gostream, pidgostream, chronic.

In case of a hospitable leak, typical for the youngest ailments, the temperature rises rapidly to 38 0Z і і vishche, vіnіkayut bіl near the slopes, there are characteristic for VKV wines of shkіri, serous membranes, vasculitis. Quickly to form on a single basis of internal organs - leg, nirok, nervous system and in. Without lіkuvannya in 1-2 the fate of the winks become insane іf lives.

When the option is most typical for VKV, it is necessary to repent from the step-by-step destruction of self-esteem, reduction of prasezdatnost. Z'yavlyayutsya b_l near the slopes. Winning shkіrnі zmіni, іnshі types, show VKV. Chvoroba is opposed to sickness during periods of sharpening and remission. The insanity of the lives of the polіorganіnі, the destructions come not earlier, not after 2-4 rocks.

In case of a chronic interruption, it is important to set the ear of VKV up. Zahvoryuvannya ovgiy hour zalishaєtsya not recognized, oskіlki manifest itself as symptoms of one of the numerical syndromes characteristic of tsyogo disease. Clinical masks of chronic VKV can be local discoid vovchak, good polyarthritis of unclear etiology, polyserositis of unclear etiology, angiospastic Raynaud's syndrome, thrombocytopenic syndrome of Verlhof, and dry Shegren's syndrome. With a wide variety of ailments, the most important picture is 10 rocky.

The flared phase of VKV is characterized by multiple symptoms of deterioration of tissue structures, vessels, and internal organs. Minimal types of diseases are characterized by three diseases: dermatitis, polyserositis, arthritis.

There are no less than 28 options for fighting shkiri during VKV. Below, a number of pathological changes of the shkiri have been introduced, which most often develop, and the appendages, mucous membranes.

· Er_thematous dermatitis of exposure. On the cheeks and the back of the nose, a stike of the erythema is formed, which will make a whiff of its own shape.

· Disco dressing. On the faces, tulips, and cuts, there are mid-range roundabouts, similar to coins, with changed edges, depigmentation and atrophic changes in the center.

· Nodular (vuzluvate) training of shkiri.

· Photosensitivity - pathological hypersensitivity of the child to sleepy insolation.

· Alopecia - generalized in the middle of the region.

· Vasculitis of sudin shkiri in viglyadi kropiv'yanka, capilaritis (dibmid hemorrhagic visip on the pads of the fingers, on the hollows, nigtovyh lodges), vyrazok in the microinfarction mice. On the face of the cannonball, the blizzard "blizzard" is a pulsating heartbeat carried over to the cyanotic vidtink.

· Erosions on mucous membranes, cheilitis

Vovchakovy polyserositis including the striation of the pleura, pericardium, inodi ocerevin.

The cutting of slopes during VKV is interconnected with arthralgia, symmetric non-erosive arthritis without deformity, ankylosis. For arthritis in vovchakov, a symmetrical reduction of other lobes of the brush, colonic loops is characteristic; It is possible to develop Jaccoux syndrome - arthropathy due to severe deformities of the lobes for the rupture of tendons, ulcers, or even without erosive arthritis. At the connection with the vasculitis, aseptic necrosis of the heads of the stegnum, brachial and other cysts does not develop very often.

Suputniy VKV mіsitis manifests itself mіalgіyu, meazovuyu weakness.

Legends and pleura are often answered. Pleural dissection is bilateral. Can be adhesive (slush), dry, exudative pleurisy. Adhesive pleurisy may not be susceptible to active symptoms. Dry pleurisy is manifested by pain in the chest cavity, the noise of rubbing the pleura. The dullness of the percussion sound, the amalgamation of the collapse of the diaphragms, is indicative of the accumulation in the pleural empty spaces of the ridin, beloved, in a small community.

Aseptic pneumonitis, which is characteristic of VKV, is manifested by an unproductive cough, bad breath. Yogo objectival symptoms do not appear as pneumonia. Vasculitis of the Legendary arteries can lead to hemoptysis, Legeny lack of blood, pitting the grip in a small number of new options for the right heart. You can thrombosis of the lung artery from the form of the infarction of the lung.

Clinical manifestations of heart pathology are typical for VKV pancarditis: pericarditis, myocarditis, endocarditis, vasculitis of the coronary arteries.

Pericarditis with VKV bowls is adhesive or dry, may be manifested by the noise of rubbing the pericardium. Onset of the diagnosis of exudative pericarditis with small accumulations of age at the pericardial emptying.

Vovchakov's myocarditis is the main cause of disturbance in rhythm, conductivity, heart failure.

Warty endocarditis of Liebman-Sachs can be supervised by multiple thromboembolisms in the inner organs with the onset of infarctions, which is the reason for the formation of heart disease. There is a lack of valves in the aortic neck, lack of mitral valve. Stenosis of valve openings is infrequently formed.

Vovchakov's vasculitis of the coronary arteries of the wikipedia ischemic ear of the heart muscle right up to myocardial infarction.

The range of young snakes in brunky is wide. Vognischevia nephritis can be asymptomatic or with minimal changes of slash siege (microhematuria, proteinuria, cylindruria). Diffuse forms of lupus nephritis can cause nephrotic syndrome with swellings, hypoproteinemia, proteinuria, hypercholesterolemia. Frequently the fight against nirokє from evil arterial hypertension. There is a large number of types of diffuse nephritis of diffuse nephritis, and there is a lack of discompensation.

Vovchak's hepatitis develops well, manifests itself as hepatomegaly, as well as impaired liver function. Wine nikoli not to produce liver failure, liver cirrhosis.

Pain in the abdomen, which is even more intense, sprains of the mucous membranes of the anterior cranial wall (vovchakovy abdominal crisis) will cause the connection with the vasculitis of the brijovy's sudines.

In most patients, there are middling and diffuse changes in the central nervous system, vasculitis, thrombosis of cerebral vessels, non-mediated immune cells of nerve cells. Types of headache, depression, possible psychosis, epileptiform attacks, polyneuropathy, rupture of rukhovykh functions.

With VKV, peripheral lymph nodes increase, splenomegaly appears, and is not associated with impaired portal hemodynamics.

Khvory VKV anemic. Often, hypochromic anemia is diagnosed, which can be attributed to a group of hallucinations. In case of immunocomplex illnesses, such as those that are present and VCB, macrophages react intensively with hemosiderin cells, such as depot deposits, vidalyayuchi (overridden) in the cystic marrow. There is a deficiency of saline for blood formation for the preservation of the mineral instead of the element in the body is not more than the norms.

Hemolithic anemia in ailments on VKV winyk with ruined erythrocytes in the process of elimination of immune complexes fixed on their membranes, as well as in cases of hyperreactivity of macrophages in cerebrovascular accidents.

For VKV typical clinical syndrome of Raynaud, Sjogren, Verlhof, antiphospholipid.

Raynaud's syndrome of obesity with immunocomplex vasculitis. For those who are sick, the ischemia of the singing dilyanoks is spastic in those who are suffering from cold or emotional stress. Rapid blink and start crooked fingers. great finger, Ridche - toes, pidboriddya, nis, wuha. After a short interval of an hour, the blight changes to crimson-cyanotic swelling, swelling of the skin as a result of post-hemic paresis of sudin.

Sjögren's syndrome is an autoimmune lesion of sleepy, lacrimal and other secretory diseases with the development of dry stomatitis, keratocon'unctivitis, pancreatitis, secretory deficiency of the mucous membrane of the slunk. In ailments, the shape of the individual can change at a link with the compensatory hypertrophy of the near-sluggish vines. Sjogren's syndrome is often associated with Raynaud's syndrome.

Werlhof's syndrome (symptomatic thrombocytopenic purpura) in case of VKV accumulation of autoimmune processes of thrombocytogenesis, great conditions of thrombocytes in the process of autoimmune reactions. It is characterized by internal petechial hemorrhages - purple. In ailments with a chronic variant of a clinical breakdown of VKV, Verlhof's syndrome can be trivial for an hour, but it is the only manifestation of a sickness. When you are in vovchaku, it is not easy to find a drop in the level of platelets in the blood is not supervised by hemorrhages. In the practice of the author of the book, the bully vipadki, if in ailments for the cob period of the BKV, the number of platelets in the peripheral blood did not eat 8-12 per 1000 leukocytes for the duration of the bleeding, so that the number of platelets in the peripheral blood was not consumed.

Antiphospholipid syndrome forms at a link with autoantitils to phospholipids, cardiolipin. Antiphospholipidnі antіla is called a vovchakovy anticoagulant. The stench is negatively injected on the stage of blood loss, the thromboplastin hour indicators are increased. Paradoxically, the presence of a Vovchak anticoagulant in the blood is characterized by a tendency to thrombosis, and not to bleeding. Slackness syndrome is manifested by thrombosis of glibular veins of the lower bones. Most of the livedo is a tree-like periwinkle on the screen of the lower bones, which can also form as a result of thrombosis of the other veins of homosexuals. In ailments on VKV, antiphospholipid syndrome is one of the main causes of cerebral thrombosis, legenevius, and pepine veins. Often present with Raynaud's syndrome.

Diagnostics

Zagalny blood analysis: a change in the number of erythrocytes, hemoglobin, in some cases, one hour because of the decrease in the value of the colore indicator (CP). In some cases, reticulocytosis appears - evidence of hemolytic anemia. Leukopenia, often rotated. Thrombocytopenia, often glib. Збільшена ШОЕ.

Zagalny analysis of the section: hematuria, proteinuria, cylindruria.

Biochemical analysis of blood: improvement in the place of fibrinogen, alpha-2-and gamma-globulin, zagalny and indirect bilirubin (with hemolytic anemia). In case of urazhennі nirok hіpoproteinemia, hypercholesterolemia, increase instead of sečovina, creatine.

Immunological allowance is allowed for positive results and low for BKV-specific reactions.

· LE-cells - neutrophils, which revenge the nucleus of a phagocytic lymphocyte in the cytoplasm. Diagnostic value of the indicator is about five LE-cells per thousand leukocytes.

· Alterations of circulating immune complexes (CVC).

· Antibody to Sm-antigen - polypeptides of short nuclear RNAs.

· Antinuclear factor - a complex of antinuclear autoantitils, specific to the lower components of the cell nucleus.

· Antibody to native DNA

· The phenomenon of a rosette is the emergence of leukocyte groups, so that the cells feel like they can lie down.

· Antiphospholipidnі autoantitila.

· Positive reaction of Coombs in hemolytic anemia.

· The rheumatoid factor is shown in the lower diagnostic credits for the turn of the swelling manifestations of the VKV.

EKG - signs of hypertrophy of the myocardium of a lithium in case of formed vads (lack of mitral and / or aortic valves), arterial hypertension of non-malignant genesis, malformations and disruption of rhythm and disruption.

Radiography of lesions - vipit in pleural discharges, secondary inflammation (pneumonitis), interstitial malignancies (legenevius vasculitis), triple infarctions in arteriosus arteriosus.

Radiography of lesions - osteoporosis without urazuvannya, ankilosuvannya.

Ultrasound pre-diagnosis: vipit in pleural empty spaces, and sometimes there is a small number of in children in black empty ones. Hepatomegaly and splenomegaly without damage to portal hemodynamics begin to appear. In some cases, there are signs of thrombosis of the liver veins - Bad Kiar syndrome.

Echocardiography - vipit at the empty pericardium, not very significant (right up to the tamponade of the heart), dilatation of the heart chambers, change in the fraction of the wick of the lone slut, dilation of the hypokinosis of the genital valve,

Ultrasound dosage of nirok: diffuse, symmetrical improvement of echogenicity of parenchyma of both organs, some signs of nephrosclerosis.

Punctuation biopsies of nirok - fail or support one of the morphological options for lupus-nephrite.

The stage of VKV activity is based on such criteria.

· I st. - Minimal activity. The temperature is normal. Little skinny. On shkiri disco oreredki. Arthralgia. Adhesive pericarditis. Myocardial dystrophy. Adhesive pleurisy. Polyneuritis. Hemoglobin more than 120 g / l. SHOE 16-20 mm / year. Fibrinogen is less than 5 g / l. Gamma globulin 20-23%. LE-cells are on the day or alone. Antinuclear factor less than 1:32. The titer of antibodies to DNA is low. The level of the CVC is low.

· ІІ Art. - Peace of mind. Garyachka up to 38 0S. Pomirne skudnennya. On the shkіrі there is a nonspecific erithema. Pidgostria polyarthritis. Dry pericarditis. Myocarditis abnormalities. Dry pleurisy. Diffuse glomerulonephritis of the low type with arterial hypertension, hematuria, proteinuria. Encephaloneuritis. Hemoglobin 100-110 g / l. SHOE 30-40 mm / year. Fibrinogen 5-6 g / l. Gamma globulin 24-25%. LE-cells 1-4 per 1000 leukocytes. Antinuclear factor 1:64. The titer of antibodies to the middle DNA. Rіven TsVK - middle.

· ІІІ Art. - maximum activity. Garyachka vische 38 0S. Virazhene skudnennya. Striking the shkiri at the viglyadi vovchakova eritemi, "blizzard" on the face, capilarite. Gostriy abo pidgostriy polyarthritis. Vipitny pericarditis. Variations of myocarditis. Vovchak's endocarditis. Vipitny pleurisy. Diffuse glomerulonephritis due to nephrotic syndrome. Gostriy encephaloradiculoneuritis. Hemoglobin less than 100 g / l. SHOE ponad 45 mm / year. Fibrinogen ponad 6 g / l. Gamma globulin 30-35%. LE-cells ponad 5 per 1000 leukocytes. Antinuclear factor is higher for 1: 128. The titer of antibodies to DNA is high. Rivn TsVK is the best one.

The American Rheumatology Association's diagnostic criteria were reviewed:

The diagnosis will be reliable if there are 4 or less pointing criteria below. For the manifestation of the least number of criteria for diagnosis, the diagnosis will be accepted (not included).

1. People's "blizzard»: Flat or fixed the erythema on the wheels, which tends to expand to the nasolabial zone.

2. Disco visip:born erythematous plaques with snug little pieces, follicular plugs, atrophic scars in the middle aged.

3. Photodermatitis:Hanging out on the shkіrі, scho z'yavlyayutsya on the shkіra of the sleepy light.

4. Erosions and turns in empty mouths:ailments of mucus empty mouth or nasopharynx.

5. Arthritis:non-erosive arthritis of two or more peripheral slopes, which can appear ailing, swollen, exudative.

6. Serositi:pleurisy, which is manifested by pleural pain, a noise of rubbing the pleurisy or signs of pleural vipot; pericarditis, which is manifested by the noise of rubbing the pericardium, internal pericardial vipotom, ekhocardiographically emerging.

7. Striking nirok:Proteinuria stance 0.5 g / dobu or more or hematuria, presence in the section of cylinders (erythrocytic, tubular, granular, minus).

8. Striking the central nervous system:Sudomy - for the duration of the drug toxicity, metabolic damage (ketoacidosis, uremia, electrolyte damage); psychosis - for the duration of the intake of psychotropic liquids, electrolyte disorders.

9. Hematological damage:leukopenia 4 10 9/ l and less, registered two or more times; lymphopenia 1.5 10 9/ l і less, registered not less than two times; thrombocytopenia less than 100 10 9/ l not pummeled ??

10. Immunological damage:antibodies against native DNA in the adjusted titre; antibodies to smooth muscles (anti-Sm); antiphospholipidnі antibodies ( adjustments IgG - or IgM - antibodies to cardiolipin, the presence of a coagulant in the blood; Hybopositive Wasserman's reaction for the presence of evidence of syphilic infection

11. Antinuclear antibodies:їх in the increased titer for the duration of the reception of the symptoms, the prevailing symptoms of the syndrome are similar.

Differential diagnosis

To spend in Persha with lupoid hepatitis (chronic autoimmune hepatitis with lateral manifestations), rheumatoid arthritis, as well as the reduction of systemic ailments with a complete tissue (Sharp's syndrome), chronic glomernimonephritis, systemic vasculitis.

Chronic autoimmune hepatitis from post-pectoral manifestations is called more common, some of the supernatant damage to internal organs, arthralgia, polyserositis, vasculitis and VK., Nagaduyuchi However, on the form of lupoid hepatitis, with VKV, the liver is affected well. Onsite massive necrosis of hepatocytes. Vovchakov's hepatitis does not go over into liver cirrhosis. On the other hand, with lupoid hepatitis following the data of punctate biopsies, there may be a slight turn and severe necrotic ear of parenchymal liver, with a further transition in cirrhosis. During the period of the formation of remission of lupoid hepatitis, the symptoms of post-pectoral lesions disappear beforehand, but I would like to have minimal signs of an ignition process in the oven. In case of systemic worm-vowchak, all navpaks are returned. Signs of the battle of the stove are extinguished before.

On the cob stages of becoming ill with VKV, rheumatoid arthritis may practically show the same clinical manifestations: feverishness, rank skutism, arthralgia, symmetric arthritis of other folds of the hands. However, in case of rheumatoid arthritis, the reduction of the slopes is more severe. Types of erosions of sloped surfaces, proliferative processes from the onset of ankylosis of a severe slope. For VKV, erosive ankylosing arthritis is not typical. Significantly difficult to establish a differential diagnosis of VKV and rheumatoid arthritis from systemic manifestations, especially on cob stages of ailments. The most common manifestation of VKV is severe glomerulonephritis, which leads to deficiency. With rheumatoid arthritis, glomerulonephritis is infrequent. In quiet people, if it is not possible to separate VKV and rheumatoid arthritis, then think Sharp's syndrome is a systemic congestion of the tissue, which is also a sign of VKV, rheumatoid arthritis, systemic sclerositis, and polymyosis.

Planning plan

· Out-of-band analysis of blood from platelet blood cells.

· Zagalny analysis of the sech.

· Test according to Zimnitsky.

· Biochemical analysis of blood: fibrinogen, gallbladder and fraction, bilirubin, cholesterol, sechovina, creatinine.

· Immunological analysis: LE-cellini, CVC, rheumatoid factor, antibodies to Sm-antigen, antinuclear factor, antibodies to native DNA, antiphospholipid antibodies, Wasserman reaction, direct and indirect Coombs probes.

· Radiography legen.

· Radiography of urazhennyh slopes.

· EKG.

· Ultrasound examination of pleural, cross-empty, liver, spleen, nirok.

· Echocardiography.

· Biopsy of the shkirno-myazovy valve (as shown - if necessary, differential diagnostics with the other systemic infestations of the resulting tissue, proof of the malignant infestation of the resulting tissue - Sharp's syndrome).

· Biopsies of nirok (for indications - for the need for differential diagnostics for other systemic discomforts of nirok, chronic glomerulonephritis).

Likuvannya

VKV tactics include:

· Applying hyperreactivity of immune mechanisms, immune inflammation, immunocomplex attacks.

· Lіkuvannya okremih clinically significant syndromes.

With the help of a change in hyperreactivity, immunity, ignition processes, glucocorticosteroids, immunodeperesanti (cytostatics), aminocholine drugs, and other methods (plasmapheresis, hemosorption) become stagnant.

On the basis of the evidence of glucocorticoid drugs є overconvolutions, it was proved that VKV was diagnosed. On the cob stages of ailments with minimal signs of activity, glucocorticosteroids, ale from non-steroidal antiapalis. It is always due to the transition of VKV, the activity of the immune-firing processes, the development of different schemes of monotherapy with glucocorticoids, combined with the use of other diseases. To fix the problem with the "overwhelming" dose of glucocorticoids with a gradual transition to the activity of the immunosuppressive process, which occurs when it is extinguished. Most often for the administration of VKV, prenizolone is used for oral administration, methylprednizolone for parenteral administration.

· In case of chronic interruption of VKV with minimal activity of immune firing, it is necessary to take oral intake of prednisolone in minimal doses, which is acceptable, - 5-7.5 mg / dose.

· In case of a state-of-the-art and state-of-the-art key transition from the II and III st. activity of VKV prednizolone is recognized at a dose of 1 mg / kg / add. Even after 1-2 days, the sick person will not turn, the dose will increase to 1.2-1.3 mg / kg / add. Take it for a stretch of 3-6 days. With a change in the activity of the immunosuppressive process, the dose should be reduced by 5 mg per day. When the dosage is 20-50 mg / day, the rate of reduction is changed to 2.5 mg per day until the minimum acceptable dose is 5-7.5 mg / day.

· In case of highly active VKV with important vasculitis, lupus nephritis, circulating anemia, leukopenia, thrombocytopenia, encephaloradiculneuritis with hospitable psychotic, cranial pulmonary disease, on the background of systematic elimination With a stretch of three days after a while, inject 1000 mg of methylprednizolone internally in droplets with a stretch of 30 quilins. The procedure can be repeated for 3-6 months. At the onset of pulse therapy, ailments are guilty of prodovzhuvati systematic oral intake of prenizolone, to eliminate any deficiency, enlargement changes in glomerular filtration.

Immunosuppressants (cytostatics) are recognized with VKV only once with glucocorticosteroid drugs, or against the background of systematic use. Immunosuppression allow the feasibility of an anti-inflammatory effect and immediately change the required dose of glucocorticoids, lowering, by ourselves, side effectsїх trivial reception. Cyclophosphamide, azathioprine, and earlier cytostatics become stagnant.

· With high activity of VKV, systemic vasculitis with widening of necrotic lesions of the skin, important pathological changes in the lungs, central nervous system, active lupus nephritis in case of unpleasantness of the dosing of glucocorticoid:

o Cyclophosphamide 1-4 mg / kg / dobu orally, abo:

o Azatioprine 2.5 mg / kg / dobut.

· With active lupus nephritis:

o Azathioprine 0.1 1 time to add internal and cyclophosphamide 1000 mg internally 1 time per 3 months.

· To improve the effectiveness of tridenous pulse therapy with methylprednizolone of another day, intravenously, 1000 mg of cyclophosphamide should be administered intravenously.

Aminoholin drugs may have additional value. The stench is recognized for trivial stagnation in case of low activity of the ignition process, chronic interruption of the VKV with overwhelming losses of the shkiri.

·

·

For the formation of blood from the blood, a surplus of autoantitils, immune complexes, mediators of the ignition process will become stagnant:

· Plasmapheresis - 3-5 procedures with one-time vision up to 1000 ml of plasmas.

· Hemosorption on active fiber and fiber sorbents - 3-5 procedures.

For the treatment of thrombocytopenic syndrome, stasis:

· immunoglobulin preparations at 0.4 g / kg / dob for 5 days;

· dinazole at 10-15 mg / kg / dob.

With the appearance of schilosti before thrombosis, low molecular weight heparin is used in 5 yew. OD for abdominal shkiru 4 here per day, antiaggreganti - chimes, 150 mg daily.

If necessary, take antibiotics of a wide spectrum of diy, anabolic hormones, sechogenic drugs, angibitorium ACE, peripheral vasodilators.

Forecast.

Unfriendly. Especially in cases of high-active lupus nephritis, cerebral vasculitis. Obviously, a sly sign of ailments with chronic, inactive interruption of the VKV. In such cases, there is an adequate liking for the disease, we have a triviality of life for 10 years.

... Systemic scleroderma

Viznachennya

Systemic scleroderma (SS) or systemic sclerosis is a diffuse disease of the resulting tissue with fibrous-sclerotic symptoms of the skin and internal organs, vasculitis of other vessels in the form of regional endarteritis.

ICD 10:M 34 - Systemic sclerosis.

M34.0 - Progressive systemic sclerosis.

M34.1 - CR (E) ST syndrome.

Ethiology.

Infected before the infection of an unidentified RNA virus, trivial professional contact with polyvinyl chloride, a robot in the minds of intensive vibration. Before becoming ill, specimens with antigens of histo-susceptibility HLA type B35 and Cw4. In the case of large ailments on the SS, there may be little chromosome aberrations - different chromatids, chromosomes and other chromosomes.

Pathogenesis

In addition to the infusion on endothelial cells of the etiological factor, the immune pathological reaction. T-lymphocytes, sensitized to antigens of progressive endothelial cells, produce lymphocytes, which stimulate the macrophage system. Have your own mother, monokini stimulated macrophages in an even larger world, they start endothelium and immediately stimulate the function of fibroblasts. Vinikak is viciously immuno-ignited colo. Postoperative stinks of oily-type dry vessels become hyper-sensitive to vasoconstrictor infusions. Formation of pathogenetic mechanisms of vasospastic ischemic Raynaud's syndrome. Active fibrogenesis in the Suddens Veda before the change in education and obliteration of the Suddens. As a result of some immunosuppressive reactions, impaired blood circulation in other vessels, an increase in interstitial tissue swelling, stimulation of tissue fibroblasts with further non-vascular sclerosis of the skin and internal organs. It is due to the nature of the immune system that there is a growing variety of options. The appearance in the blood of antibodies up to Scl-70 (Scleroderma-70) is linked to the diffuse form of CC. Antibody to centromere є typical for CREST syndrome. Nuclear antibodies - for scleroderma lesions of nirok and overlap syndrome with dermatomyositis-polymyositis. It is interrupted by that diffuse form of the SS pathogenetic sutta to argue:

· Bordered (limitovana) form SS vidoma yak CREST-syndrome. Yogo marks є calcification ( Calcinosis), Raynaud's syndrome ( Reynaud), disruption of peristalsis in stravohode ( Esophageal motility disorders), sclerodactylia ( Sclerodactilya), teleangiectasia ( Teleangiectasia). Characteristic pathological changes are the head rank of the shkiri of exposing the fingers of the hands distally behind the metacarpophalangeal slope. The price is usually good enough for getting sick. The reduction of internal organs is rather rare and there is no more in case of a trivial outbreak of ailments, and as soon as they get sick, then it is easier to pass, less in case of diffuse forms of SS.

· The diffuse form of SS (progressive systemic sclerosis) is characterized by sclerotic symptoms of the sclerosus of the upper protuberances of the proximal pyastophalangeal lobes, the small dylyanoks of tila, right up to the entire surface. The details of the internal organs are determined earlier, not for the interconnected form. The pathological process involves more organs and tissue structures. Especially often and it is important to fight for nirkah and legends.

key picture

Zahvoryuvannya can be countered in hospitality, food, chronic forms.

The gostra form of a diffuse SS is characterized by a shimmery stretch of less than one rock development of all stages of a shkiri defense. Immediately, it’s reaching its culmination development of internal organs, in front of a nyrok, a leg. By extending the period of the ailment, the maximum visualization of the indicators of the external, biochemical analysis of the blood appears, and demonstrating the high activity of the pathological process.

When a gostroma breaks down, it grows up at a normal pace, even with the presence of all typical for a diffuse SS the level of shkiri, vasomotor breakdowns, the level of internal organs. The visualization of laboratory and biological indicators is indicated, so that the activity of the pathological process can be visualized.

Chronic stretching of the SS is characterized by a step-by-step ear, depending on the progress that lasts for an hour. The most common form is the form of ailment - CREST-syndrome. The clinically significant lesion of internal organs, the appearance of laboratory and biochemical indicators, is not very important. In an hour, the ailments may have symptoms of hypertension of a small cola, accumulated by an obliterated endarter of the Legendary artery and hylok, signs of fibrosis of the leg.

In typical SS problems, it is necessary to fix pathological changes in the school. It will help me to have a painful taunting of the fingers of both hands (nabryakova phase). Let the shkіra go down (indurative phase). Sclerosis

The sclerosed skin becomes smooth, shining, taut, even dry. It cannot be folded, fragments of the won are soldered with fascias, oxystyanoy, periarticular structures, which are suitable for use. She's got a little hair. Nigty deform. On the vitality of the hands, it is easy to recognize and generally fear traumatic ears, spontaneous movements, and gnats. There are teleangioectasis.

It is not possible to get lost in the way that is typical for the SS against the shkiri of denunciations. Exposing an old man to be amic, mask-like, unnaturally bland, unreasonably pigmented, often with crimson middles to teleangiectatu. Let's get stuck at the bird's eye view. There are “goodies” looking, splinters of a sclerotic banner of a shkiri chola and a wide open wide shchilini, an accelerated morgan. The mouth's crack is sounding. Shkіra navkolo rota squeeze into the shape of the radial folds, but do not straighten out, nagaduyuchi form of "pouch".

With the limitation of the SS form, the battle is interrupted by the deprivation of the shkіroy fingers of the hands and face. With diffuse forms of swelling, inductive-sclerotic snakes step by step expand on the chest, back, legs, everything is dark.

Exercising the chest and the back of the sash at the sickly view of the corset, which will entangle the spirited ruffs of the breast cage. The total sclerosis of the shrunken crooked forms the picture of the pseudo-communion of a sick person is the phenomenon of “living relics”.

One hour, with shkirny covers, you can get rid of the mucous membranes. It is not easy to think of the dryness that they have, the visibility of the slime in the mouth, the tear in the eyes, the unhappiness of the poster. Quite often, it’s worth noting about the formulation of “dry” Sjogren's syndrome in a sick person on SS.

At the same time with the nabryako-indurative changes of the school, and in the vicinity of the fall and up to the shock of the school, an angiospastic Raynaud's syndrome can be formulated. Someone who is sick is fixing turbuvati on the attack of raptus blunder, oniminnya of fingers, on the other hand, on the nose, in the cold, on the smallest emots, and to navigate for no apparent reason. The bitterness of disagreement will turn into a spasmodic hypothesis; Disease of Raynaud's syndrome

Suglobovy syndrome is carried to early manifestations of SS. Wien can be interconnected with polyarthralgia without affecting slopes and navkoloslobovyh structures. In some cases, there is a symmetrical fibrous scleroderma polyarthritis of other slopes of the hands with scargs on the skin and bile. For a new type of exudative, and sometimes proliferative changes in rheumatoid arthritis. Scleroderma pseudoarthritis may also form, which is characterized by interdigitated crumbling of slopes, due not to lesions of sloped surfaces, but to the enlarged capsule of the slope and tendon of the joints with an inductively scaly-toothed apex. Frequently, lumpy syndrome will collapse with osteolysis, shortening of the fingernose phalanges of the fingers - sclerodactylation. The syndrome of the carpal canal can be formed with parasthesia of the middle and viable fingers of the hand, pain, which grows up the hill before the start, and the initial contracture of the hand.

Myazova weakness in diffuse form SS. The reasons for this are diffuse atrophy of the mucous membranes, non-firing muscular fibrosis. In some cases, there is a manifestation of fiery myopathy, which is identical to that in ailments for dermatomyositis-polymyositis (perekhresny syndrome).

Pediatric calcification appears as a head rank in case of SS lymphoma (CREST-syndrome), and deprivation in a small number of ailments with a diffuse form of illness. Calcifications often grow in nature traumatization - fingers of the hands, the surface of the fingers, colin - Tib'erzhe-Weissenbach syndrome.

Damage to the structure of the wall and motor function of the stratohover during the SS. In ailments on the SS, the smooth muscles of the lower third of the strain are replaced by collagen. The musculature of the upper third is transversely smugasta. Vinnikє stenosis of the lower ducts in the duct and compensatory enlargement of the upper ones. The structure of the mucous strain is changing - the metaplasia of Beretta. As a result of gastroesophageal reflux, erosive reflux-esophagitis often occurs, it develops in a pathway to the stravohode, posttrazovaya strictures stravochidno-shlunkovogo sputus. Mole atony and dilatation of the slunk, two-fingered intestine. With the diagnosis of diffuse fibrosis of the shlunk, it can be damaged due to the formation of sideropenic syndrome. Atony, dilatation small intestine... Fibrosis of the small intestine wall is manifested by malabsorption syndrome. Disorder of the intestine can lead to diverticulosis, manifested by constipation.

In ailments with a limited form of seizure in viglyadі CREST-syndrome, one can form first bilarial cirrhosis of the liver, the first symptom of which can be "unreasonable" itching of shkіrnykh curvatures.

In ailments with diffuse SS, there is a lesion in the basal viglyad, and in the case of diffuse pneumofibrosis, it manifests itself as a progressive deficiency. It is a good idea to skarzhayutsya for a post-layover, so that you can do your best for physical activity. There may be dry pleurisy due to pain in the chest cavity, a noise of rubbing the pleurisy. In ailments with a limited SS in the form of an obliterated endorsement of the Legendary artery and of the Gilok Wininikov Legeneva hypertension with the overwhelming of the right heart.

The diffuse form of the SS is inodi to accelerate the battle of the heart. Myocarditis, myocardial fibrosis, ischemia of the myocardium is enriched with irregular vasculitis of the coronary arteries, fibrosis of the stools of the mitral valve due to the form of this deficiency can cause decompensation of hemodynamics.

Striking nirok is attracted by the diffuse form of the SS. Pathology of nirok is a free alternative to Raynaud's syndrome. Scleroderma nyrca is characterized by a decrease in sudins, glomeruli, tubules, interstitial tissues. For clinical manifestations of scleroderma, nirka does not lead to glomerulonephritis, which is the opposite of arterial hypertension, sech syndrome in vigilance proteinuria, hematuria. Progressive decrease in glomerular filtration should be made up to HNN. As a result of obliterated fibrosis of the middle arteries in the midst of a short period of time (hypothermia, hemorrhage, etc.), cortical necrosis can be seen due to the lack of hospitals - sclerosis.

The breakdown of the nervous system is accompanied by an obliterating vasculitis of the cerebral arteries. Spastic attacks from the lesions of the internal cranial arteries, as one of the manifestations of Raynaud's syndrome, may suffer from severe attacks, psychosis, passing hemiparesis.

The diffuse form of the SS is characterized by a lesion of the thyroid gland in the viglyad of autoimmune thyroiditis, fibrous atrophy of the organ.

Diagnostics

· Zagalny blood analysis: maybe normal. Some signs of anemic hypochromic anemia, low leukocytosis or leukopenia. Buvaє zbіlshenoyu SHOE.

· Zagalny analysis of the slaughter: proteinuria, cylindruria, microhematuria, leukocyturia, with CNI - a decrease in the slice part. Zbіlshena ekskretsіya oskіprolіnu - sign of collagen impaired metabolism.

· Biochemical analysis of blood may be normal. The active process of supernatant growth is replaced by fibrinogen, alpha-2- and gamma-globulin, seromucoid, haptoglobin, oxyprolin.

· Immunological analysis: specific autoantitila up to Scl-70 with diffuse forms of SS, autoantitil up to centroids with reduced forms of sickness, nuclear antibodies with severe nirocositis, transcressive syndrome of SS-dermatomatomy. Most of the ailments have a rheumatoid factor, in some cases there are only one LE-clitini.

· Biopsy of the shkirno-myazovy valve: irregular vasculitis of other vessels, fibrous-sclerotic changes.

· Puncture biopsies of the thyroid gland: morphological signs of autoimmune thyroiditis, vasculitis of other sudins, fibrous arthrophy of the organ.

· X-ray dosage: calcification in tissues of the fingernose phalanges of the fingers, lactose, colony lobes; osteolysis of the distal phalanges of the fingers of the hand; osteoporosis, ulceration of the slopes, one of the ankylosis of the slopes. Breast cell - interpleural adhesions, basal, diffuse, infrequently cystic (in some cases) pneumofibrosis.

· EKG: signs of myocardial dystrophy, ischemia, high-grade cardiosclerosis with impaired conductivity, malnutrition, hypertrophy of the myocardium in those who are in front of the heart with a deficiency of the mitral valve, which has been formed.

· Echocardiography: verification of the metral wadi, damage to the speedy function of the myocardium, dilatation of the chambers of the heart, there may be signs of pericarditis.

· Ultrasound diagnostics: structural signs of bilateral diffuse lesions of nirok, characteristic of nephritis, evidence of autoimmune thyroiditis, fibrous atrophy of the thyroid gland, and some other signs of bilious liver cyrosis.

CLINICAL CRITERIA OF THE AMERICAN Rheumatological Association for the Development of Systemic Scleroderma:

· "Great" criteria:

o Proximal scleroderma - bilateral, symmetric sweating, shrinkage, induration, sclerosis of the dermis of the fingers, the shkiri of the fingers proximally from the palatal-phalangeal and metatarsal-phalangeal halls of the loamy loam

· "Mali" criteria:

o Sclerodactylia - induration, sclerosis, osteoliasis of the endov phalanges, deformity of the fingers of the hands;

o Scars, tissue defects on the pads of the fingers of the hands;

o Basal legenevium fibrosis from two sides.

For the diagnosis of SS, a sick person may be either "great", or two "malikh" criteria.

Clinical and laboratory signs of the activity of the inductive-sclerotic process in ailments on the SS:

· 0 tbsp. - Visibility of activity.

· I st. - Minimal activity. Pomirni trophic damage, arthralgia, vasospastic Raynaud's syndrome, SHOE up to 20 mm / year.

· ІІ Art. - Peace of mind. Arthralgia and / abo arthritis, adhesive pleurisy, symptoms of cardiosclerosis, SHOE - 20-35 mm / year.

· ІІІ Art. - Visoka activity. Garyachka, polyarthritis with erosive lesions, high-grade or diffuse cardiosclerosis, lack of a mitral valve, scleroderma nirka. SHOE change 35 mm / year.

Differential diagnosis

It is carried out in Pershuyu with middle scleroderma, other diffuse diseases of the whole tissue - rheumatoid arthritis, systemic worm vovchak, dermatomyositis-polymyositis.

Razrіznyayut plaque, kraplepodіbnu, kіlcepodіbnu, line form of the middle (micevoy) scleroderma. On the basis of limited and diffuse forms of SS in case of moderate scleroderma before the pathological process, it is not possible to twist your fingers and face. Systemic manifestations of a little or no for a trivial overload of ailments.

Rheumatoid arthritis and SS are lighter in case of formulated in ailments on SS, pseudoarthritis in vigilia with pseudoarthritis and sclerotic lesions of periarticular curvatures. Radiologic in vipads of mild striations of the slope itself. However, with SS, and with rheumatoid arthritis, there can be symmetric polyarthritis of the other joints of the hands with a characteristic skutism, tendency to ankylosing. For such conditions, the differentiation of the disease on SS acne addictive to the development of symptoms of an indolent, and then of a sclerotic lesion of the fingers of the hands, exposing, and in the case of diffuse forms of the SS - a school of children of the age. SS is characterized by lesions (pneumofibrosis), which is not common in patients with rheumatoid arthritis.

Differential diagnostics of systemic worms by Vovchak is based on the detection of SS-specific tests for the curvatures. When you wake up on the SS, polyarthritis is good, do not lead to deformities, ankylosing loam. Vovchakov's pseudoarthritis - Jaccoux syndrome - arthropathy with stiff deformities of the joints for the rupture of the tendon, ulcers. Vona protects without erosive arthritis. There is a form of scleroderma pseudoarthritis on the outskirts of the swollen bag with an inductively changing abnormally sclerotic skin over the slope. The diffuse form of anemic disease can be identified as a systemic cervical virus due to the presence in the blood of SS-specific autoantitils to the Scl-70 antigen.

For SS, on the form of dermatomyositis-polymyositis, characteristic indurative and sclerotic lesions of the shkiri, secondarily, myopathy is also common. With dermatomyositis-polymyositis, there are high indicators of creatine phosphokinase activity in the blood, which are not common with classical SS variants. Also, there is a shortage of symptoms of SS with signs of dermatomyositis-polymyositis, followed by a diagnosis of overlap syndrome of systemic damage to the resulting tissue.

Planning plan

· Zagalny blood analysis.

· Zagalny analysis of the sech.

· Instead of hydroxyproline in the section.

· Immunological analysis: autoantitil up to Scl-70, autoantitil up to centromeres, antinuclear antibodies, rheumatoid factor, LE-cellini, CVC.

· Biopsy of the shkirno-myazovy valve.

· Thyroid biopsies of the thyroid gland.

· Radiologic dosage of hands, lesions, knee slopes.

· Chest x-ray.

· EKG.

· Echocardiography.

· Ultrasound dosage of organs in the black empty, nirok, thyroid zone.

Likuvannya

The tactics of transferring the attack on the offensive injections on the body of the sick person:

· Galmuvannya activity of obliterated endarition of other vessels, sclerotherapy of sclerosus, fibrosis of internal organs.

· Symptomatic treatment of pain (arthralgia, mialgia) and other syndromes, impaired functions of internal organs.

For the suppression of excess collagen in ailments with an active ignition process, during the pre-construction flow of the SS, it is indicated:

· D-penicylamine (cuprenil) orally at 0.125-0.25 per dose every other day. In case of ineffectiveness, the dosage should be increased to 0.3-0.6 per doba. As soon as D-penicylamine is used, it will lead to the appearance of shkirny visipans, and the dose should be changed to add prenizolone - 10-15 mg / add vseredin. Appearing on aphids of such a growth of proteineuria є as a result of the repeated use of D-penicylamine.

To reduce the activity of mechanisms in the synthesis of collagen, especially in the case of ineffectiveness, for example, there is no evidence for D-penicylamine, it is possible to use:

· colchicine - 0.5 mg per dobu (3.5 mg per day), with incremental dose adjustments to 1-1.5 mg per dobu (close to 10 mg per day). The drug can be taken with a sweep of the second-choice rock_v post.

In case of diffuse forms of SS with swelling and severe systemic manifestations, there is a dose of immunosuppressive doses of glucocorticoids and cytostatics.

· prednizolone orally at 20-30 mg before reaching the clinical effect. Then the dosage of the drug can be changed to an acceptable level - 5-7.5 mg / add, and it is recommended to take it for 1 time.

For the daytime efect, viniknennya side reactions at the reception of large doses of glucocorticoids, cytostatics vikoristovuyu:

· Azathioprine orally 150-200 mg / dob for oral administration 15-20 mg / dob prednizolone for 2-3 months.

In case of a chronic SS leak, it is important shkіrnymi manifestations, minimal activity of the fibrosing process, next to the sign of aminochinolin preparations:

· Hydroxychloroquin (Plakvenil) 0.2 - 1-2 tablets per day for 6-12 months.

· Chlorohin (delagil) 0.25 - 1-2 tablets per day for 6-12 months.

Symptomatic signs are used in advance for compensation of vasospastic reactivity, treatment of Raynaud's syndrome and other vertebral lesions. At the same time, blockers of calcium channels, angibitoria ACE, antiaggreganti:

· Nifedipin - up to 100 mg / dob.

· Verapapil - up to 200-240 mg / dob.

· Captopril - up to 100-150 mg / dob.

· Lisinopril - up to 10-20 mg / add.

· Curantil - 200-300 mg / dobu.

In case of suglobovy syndrome, preparations are indicated from the group of non-steroidal anti-pyretic agents:

· Diclofenac sodium (ortofen) 0.025-0.05 - 3 times a day in the middle.

· Ibuprofen 0.8 - 3-4 times a day in the middle.

· Naproxen 0.5-0.75 - 2 times a day in the middle.

· Indomethacin 0.025-0.05 - 3 times a day in the middle.

· Nimesulid 0.1 - 2 times a day in the middle. The drug is selectively for COX-2, and it can also be stagnant in ailments on an erosive-inflicting injury to the intestinal tract, a shlunk and two-fingered intestine, which is nonselective non-steroidal anti-epileptic drugs are contraindicated.

For muscular treatment, it is possible to get 25-50% of Dimexidum solutions at the viglyadі aplіkatsіy for urazhenі dіlyanka shkіri for 20-30 hviliya - up to 30 aplications for the course of treatment. Indications of sulfated glucosamine in ointments. It is possible to fix the lidaz with a path of internal equipment, electrophoresis, phonophoresis in the inductive change of the school.

Forecast

It appears as a pathomorphological variant of ailment. For a limited form, the forecast is to reach a friendly one. In case of diffusional form, lay it down as a development and decompensation level of nirok, legen, heart. Nowadays that adequately lіkuvannya sutta prodovzhu life of the ailments on the SS.

4. Dermatomyositis-polymyositis

Viznachennya

Dermatomyositis (DM) or dermatopolymyositis is a systemic fiery disease due to substitution of tissue tissue with fibrous structures and tissue damage to the pathological process of skeletal and smooth muscles, shkiri, other vessels. For the duration of the day, shkiri vikoristovuyut term polymyositis (PM).

ICD 10:M33 - Dermatopolymyositis.

M33.2 - polymyositis.

Ethiology

The ethiological factor DM-PM can be latent infection with carniviruses, some viruses of the Coxaci group, which are introduced into the genome of oleaginous cells. The association of DM-PM with low puffy processes can be indicative of either the cinnamon of the virus etiology of the puffs, or the demonstration of the antigenic mimicry of puffy structures and meat tissue. Before becoming ill, individuals that may have antigenicity of histoxicity HLA type B8 or DR3.

Pathogenesis

The launch of pathogenetic mechanisms of incapacitation in infectious and genetically sick individuals may be in the presence of nonspecific conditions: hypothermia, excessive sleepiness, vaccination, hospitality and toxicity. ratios of antigenically spore-like cell populations. The inclusion of microphage mechanisms in the elimination of the organisms of immune complexes by activation of the processes of fibrogenesis, suppression of the systemic firing of other vessels. At the link with the hyperreactivity of the immune system, which is directly related to the destruction of the internal nuclear positions of the virus, the blood contains antibodies Mi2, Jo1, SRP, autoantitila to the nucleoproteins of the genetic nuclei.

key picture

Ingestion can be counteracted in hospitality, food preparation and chronic forms.

The gostra form is characterized by the rapid appearance of a fever iz from a temperature of up to 39-40 0C. Immediately, there are pains, weakness in the muscles, arthralgia, arthritis, and urinary tract infection. Generalized training of all skeletal muscles is rapidly developing. Shvidko is progressing myopathy. In a short period of time, an hour's ailments will practically increase our anxiety. Winikayut heavy destructive kovtannya, dikhannya. It appears and rapidly decompensates the battle of internal organs, in front of the heart. The triviality of life during the hospitality of the form does not change for 2-6 months.

Pidgostry is characterized by being visible at the sick ear of the ear. There is evidence of mіalgії, arthralgia, and step by step the growth of myase is weakness. Sleepy insolations form a characteristic feature on the faces, on the critical surfaces of the breast tissue. There are signs of internal organs. Outside the growth of the clichéd picture, the lethal blight comes in 1-2 fate.

The chronic form is known to be good, cyclical from trivial periods of remission. The whole variant of ailments can be rarely brought to a nasty death, intertwined with intermittent, uncommonly local atrophic and sclerotic changes of mucous membranes, curvatures, softly swirling myopathies, compensated internal changes.

Myazova pathology є I know the DM-PM. I will inevitably have a progressive weakness in myself, so that I will start to supervise myself with miracles of higher intensity. When there is an active delayed test, the testicular mucus for a rakhunok nabryak, with a decrease in tone, soreness. In an hour, the patients were diagnosed with a pathological process of mucus changes as a result of atrophy and fibrosis.

Move towards the proximal group of cystic mucus. Distal groups of hand muscles that will get better.

Filling that fibrosis of the breast tissue, with diaphragms, will ruin the ventilation of the lungs, causing hypoxemia, creating a grip in the legendary artery.

Striking the transversely dark musculature of the pharynx and the proximal direction to the ventricular tract will destroy the processes of kovtannya. Ailments are easy to choke on. Rіdka їzha can drive krіz nіs. The sound of the larynx is striking with a snarling voice, like an old hoarse voice with a nose-like timbre.

Okorukhovi, zhuvalny, іnshі m'yazy of accusation, you don’t want to fight.

Pathological changes in small curves are characteristic of DM and the need for PM. Possible advancing options for fighting shkiri:

Get the fabric on є to finish with a rare pathology. The clinical picture of the whole ailment is characterized by some signs of colagenous ailments. The pathology of the disease is called Sharp's syndrome. Most often, such a symptom complex is indicated in the pubertal period and in patients of the middle age. In the case of the zanedban form of pathology, it can lead to serious ones that are not safe for the life of the inheritance. In the statistic, the symptoms of the loss of the resulting tissue are clearly visible.

Sho tse take

In the past, the pathology was even more important to diagnose. Andzhe signs of Sharpe's syndrome can show manifest rheumatic ailments. It’s not surprisingly long ago that the disease was described as autoimmune deterioration.

With a decrease in the resulting tissue (SZST), the patient has some signs of advanced rheumatic pathologies:

• dermatomyositis;
• scleroderma;
• rheumatoid arthritis;
• polymyositis.

The patient does not necessarily have a basic picture of all the disease ailments. There are several symptoms, lingering autoimmune pathologies.

Code z MKL

According to ICD-10, the change in the resulting tissue is seen in the vicinity of the group of pathologies with the code M35 (Інші in the resulting tissue). The external code of the SZST is M35.1. Perekhresny rheumatic syndromes are included in the whole group. The word "perekhresnі" means, for a given pathology, signs of ailments from the resulting tissue (collagenosis) are meant.

Reasons

In the past, the exact causes of Sharpe's syndrome are not clear. Zmіshane ailment with a lucrative fabric has an autoimmune character. This means that the immunity of people for unrecoverable reasons to start attacking the health of the cell.

How can you provoke this kind of robotic force into an organism? Doctors allow it to be infused into the robot immunity can be trivial by taking a lot of people from lykarskie. A great role is played in the diagnosis of autoimmune reactions in the development of hormonal impairment and in the recovery of the endocrine system. The reasons for the FTA are not often attributed to children and women in the period of climax.

Negative emotional background can also be found on the robotic immune system. The psychosomatics of a wicked affliction with a good fabric is tied up with serious stresses. This pathology is often identified in people who are sick before depression, as well as in people with neurosis and psychosis.

Name it in people who may have a decline in mental capacity to rheumatic ailments. The inflow of unwelcoming bureaucrats є deprived of the descent mechanism of the detection of autoimmune diseases.

Symptoms

The change in the affliction of the resultant tissue is contrary to the chronic form and without the practice of step-by-step progress. The pathology is systemic, the enemy is not deprived of the shkiru that suglob, but the whole organism.

Even more often on the cob, I get sick of old damaged blood circulation in the fingers of the hands and nig. Tse nagaduє show Raynaud's syndrome. Through the spasm of the sudin, people blink and cool fingers and nig. Warm up the skin on the hands and feet of the swollen bluish appearance. Cooling of the pictures of supravolume is swelling with pain syndrome. Such spasms of the sudin can cause the wines of rock to develop until they are signs of ailments.

Most patients have more pain in the slopes. The fingers of the hands are strongly swollen, the ruins become sore. The weakness of the mucus is indicated. Through the beating and swelling of the child, the patient grows up to grasp the fingers and wipe out the small objects in his hands. It is similar to the cob to show rheumatoid arthritis, for with a lot of severe deformities of the cysts. Before a pathological process, one can get one of the joints, most often the number of cases.

Nadal from the people they win the hearts and beads on the skirts, especially in the area of ​​the brushes and faces. To rinse the gorges of the dilyanki m'yaziv, yak at Shkirni pokrivi to vandalize, in some cases there are vyrazki on epіdermіsі.

The patient has a step-by-step self-respect. Bіl at the slopes and shkіrni visipannya supervised by offensive symptomatology:

• backward weakness;
• seeing scooters at the slopes of a dream;
• sensitivity to ultraviolet;
• dry slime company that difficult kovtannya;
• vipadannya hair;
• unreasonable decrease in vagi during normal eating;
• temperature adjustments;
• for improvement of lymph nodes.

In zanedbani vipadkah pathological process extends to nirki and legeni. Winikak glomerulonephritis, to move in the place of protein in the sech. Patsinti skarzhivayutsya on the beads at the breasts that complicate the action.

May be accelerated

Zmіshane ailment with a favorable fabric є an unsafe pathology. If there is a pathological process of twitching internal organs, then in case of an inaccurate lykuvannya there may be such acceleration:

• nirkova lack of;
• insult;
• firing the mucous membrane to the stravohode;
• digging through the intestines;
• myocardial infarction.

Such an acceleration is indicated in case of an uncomfortable interruption of ailments that during the day of proper therapy.

Diagnostics

A doctor-rheumatologist is involved in the treatment of the SZST. Symptoms of a malignant infestation with the resulting tissue are even more malignant and may show some other pathologies. Through the price, it is difficult to find out the difficult diagnostics of the production.

Patients should be given a serological analysis of blood for antibodies to nuclear ribonucleoprotein. As long as the indicators of the long-term progress are permissible, and if the ailments are diagnosed with arthralgia and Raynaud's syndrome, then the diagnosis will be susceptible to susceptibility.

Dodatkovo mean the following:

• clinical and biochemical analyzes of blood and slaughter;
• doslіdzhennya urini from Nechyporenka;
• analysis for rheumatoid factor and specific immunoglobulin.

If necessary, an ultrasound scan is performed, as well as a radiography and an ehocardiogram.

Likuvannya method

Likuvannya of mischievous disease with the resulting fabrics are straightened in front of the suppression of the autoimmune reaction. Patients recognize the following:

1. Corticosteroid hormones: "Dexamethasone", "Metipred", "Prednizolone". The preparation is to change the autoimmune reaction and heat in the slopes.
2. Cytostatics: "Azatioprin", "Imuran", "Plakvenil". Such liquids can also be adapted to the robot of the immune system.
3. Non-steroids of anti-fiery action: Diclofenac, Voltaren. Їх priznachayut in case of swelling pain and swelling of the slopes.
4. Calcium antagonists: "Verapamil", "Diltiazem", "Nifedipin". Tsi preparations are used to protect the heart-vascular system.
5. Іngіbіtory proton pump: "Omeprazole". We are sick of Sharpe's syndrome to take medication for an hour or so, and for an hour or so. It can negatively affect the organs of the intestinal tract. The drug "Omeprazole" will help cleanse the mucous membrane of the slurry from an aggressive injection of liquor.

It is also more complex to avoid stinging ailments and allows you to reach the stage of remission.

It is important to remember that drugs for the treatment of SZST are often reduced immunity. These patients need to protect themselves from contacts with infectious ailments and overcooling.

Forecast

Does Chi infuse Sharpe Syndrome on the triviality of life? The prognosis of the sickness is cleverly friendly. Inadequate damage to the internal organs with SZST develops earlier, but not for other autoimmune pathologies. The fatal result is indicated only in case of advanced forms of ailments and the appearance of acceleration from the side of the heart and nirok.

However, it’s a memory trail, but it’s a chronic affliction, and it’s not going to grow up until the end. Often, patients are shown pre-intake of medications. Iakshcho ailments to meet the recommended scheme of treatment, the prognosis of ill health. Timely therapy for additional help to improve the normal quality of the patient's life.

Prevention

The specific prophylaxis of the disease has not been broken up, and no evidence of the exact causes of autoimmune pathologies has been established. Lykar-rheumatologists please follow the next recommendations:

1. Slid unique uncontrolled intake of medicines. A trivial course of drug addiction can be done without an obvious drug.
2. In case of a decline in scholasticity to autoimmune pathologies, a unique development of sleepy light is regularly taken and a prophylactic examination by a rheumatologist.
3. It is even more important due to the possibility of unique stress. Emotionally labile people need to take sedative drugs and consult a psychotherapist.
4. In case of a diagnosis of pain in the slopes of the symptoms and spasms of the peripheral vessels, it is necessary to turn up before the doctor and go through the treatment.

Tsi come in to help reduce the diagnosis of autoimmune rheumatic pathologies.