Digital virazki with scleroderma. Raynaud's phenomenon, digital creep and critical ischemia in systemic scleroderma: take a look at the problem and management

Systemic scleroderma, or progressive systemic sclerosis, is referred to a group of autoimmune systemic igniter get sick with good fabrics... Vona is characterized by a stadial transition and a great polymorphism of clinical manifestations, connected with the characteristic problems of children, acts of internal organs and support-arm apparatus.

The basis of the distribution is wider than the cascade of destruction of microcirculation, ignition and generalization of fibrosis. The triviality of life with systemic scleroderma is due to the nature of the transition, the stage and the overwhelming lesion of the organs and systems of the organism.

Illness and illness

According to the average statistical data, the primary disease rate in 1 year per 1,000,000 population is 2.7 to 12 per capita, and the broader range of pathology is from 30 to 450 per capita per 1,000,000 population. The growth of ailments is possible in young groups of children, including the middle of the young (juvenile scleroderma).

However, the ear is most often indicated in the period from 30 to 50 years, I want, with a detailed introduction of the ear, signs of rarely occurring in more early vikah... The female ailment is hostile (for winter tributes) 3-7 times more often, in proportion to the choloviks. Less state of the art is seen in the statistics of the incidence of the middle of children and middle-aged adults, for which there are 45 years of age.

Retrospective data on the vision of ailments (to live), in the fallowness of the options for overcoming ailments, and with a natural development, to show the onset of vitality:

• in case of a gallant, rapidly progressing interruption in the growth of tissue fibrosis and cob symptoms in viglyadas, the triviality of life does not change 5 years, with a total vision to become deprived of 4%;
• in case of pidgostroma, along with a progressive overloading of the immune system, with cob symptoms in viglyadi of the angular syndrome; The triviality of life can become up to 15 years, with a total vision in the first 5 years - 75%, 10 years - close to 61%, 15 years - in the middle 50%;
• with a chronic, progressively progressive overloading of sudin pathology with cob signs in vigilia Raynaud's syndrome; Living in the first 5 years of ailments - in the middle 93%, 10 years - close to 87%, and 15 years - 85%.

Ethiology and pathogenesis of illness

The reasons for the development of systemic scleroderma are insufficient. It’s an hour to get involved in the day, so won’t be rich in factories, let’s summarize:

1. Genetic schism, surrounded by mechanisms, which are already deciphered. An association was revealed that there was an association with some antigens of tissue confusion, diseases of family manifestations with specific autoantibodies, etc.

2. The inflow of viruses, in the middle of which the main inflow of cytomegalovirus and retroviruses is visible. I also respect the role of the active latent viral infections, The phenomenon of molecular mimicry and іn. Remain in product immune system humoral antibodies, which reduce antigens from the statements of immune complexes, as well as in the development of cellular toxic T-lymphocytes. Smell to ruin the cells of organism, in which there are viruses.

3. Influence of exogenous and endogenous factors in rhizic. It is especially important to hope for:

• overcooling and frequent and trivial getting over sleepy interchanges;
• vibratsii;
• industrial flint saw;
• chemical agents of industrial and by-channel operations - bets on the recycling of naphtha products, chlorinyl, pesticides, organic retailers;
• deyakykh gruzovykh products, scho to revenge rapeseed oil, i food additives s L-tryptophan;
• Implants and okremim medicinal drugs, for example, bleomycin (anti-inflammatory antibiotic), vaccines;
• neuroendocrine damage, part of stress stuns, schilnosti up to vermin spastic reactions.

Schematic wiklade of the folding mechanism for the development of the disease

Typical rice systemic scleroderma є overwhelming fibroblasts of the gene protein. In the norm, the process of making new fabrics is renewed and replaced with a scar (sclerosis, fibrosis).

In case of autoimmune semi-tissue diseases of physiology in the wicked minds, the minds of the minds overwhelmingly accept, swell pathological forms... As a result of damage, it is normal that tissue is replaced by scar tissue, as well as damage to skin curves and wrinkles in the slopes and organs. The initial scheme for the development of the process is advancing.

Viruses and factors of risk on aphids of genetic strength are infused on:

1. Full-tissue structures, which can lead to a defect in cell membranes and an advanced function of fibroblasts. As a result of a large amount of oversized production of collagen, fibrokinetin (the great glucoprotein to the matrix), proteoglycans and glucose aminoglycans, which are folding bottles Before which immunoglobulins (antibodies) are known, there is a large proportion of blood hormones, interferon and other.
2. Microcirculatory bed, as a result of which the endothelium is formed Price, in its turn, to produce up to the growth of myofibroblasts (cells, similar to other fibroblasts and smooth cells at once), deposition of platelets in other vessels and adhesion (adhesion) on vessels and impaired penetration of the rest.
3. The immune system of the body, leading to an imbalance of T- and B-lymphocytes, takes a part in the formulated immune responses, as a result of which the function of the first is impaired and others are activated.

All tsi factories, in their own heart, bring fake sales offensive porushen:

• Superfluous establishment of collagen fibers with advanced progressive generalized fibrosis in the dermis, support-rocking apparatus and internal organs. Fibrosis is the growth of the resulting fabric.
• Over-surplus production of collagen cells in the walls of ankle vessels, tightening of the basal membranes in them and pulmonary fibrosis, increased laryngeal disease and thrombosis in other vessels, and tightening of their health. All the time until the destruction of other vessels with the development of convulsive spasms due to the type of Raynaud's syndrome and destruction of the structure and function of internal organs.
• Improvement of the awareness of cytokines (specific peptide and information molecules), immune complexes and autoantitils, as well as to produce up to the burning of the inner shell of other vessels (vasculature), but also of other organisms.

In such a rank, the main Lankans of the pathogenetic lantsyug є:

• destruction of mechanisms of cell and humoral types of immunity;
• defeat of other vessels with the destruction and distribution of the function of the endothelium of the judgment;
• Disruption of the processes of establishment of collagen cells due to the promotion of the formation of smooth fibers and collagen, so that fibrous extra-nutritional tissue of organs and systems with damaged functions is manifested.

Classification of systemic scleroderma and short characteristics of the surrounding forms

When the diagnosis is formulated, the signs of systemic scleroderma are concretized according to such characteristics as the clinical form of the disease, the variant of its current and the stage of development of pathology.

Razr_znyayut such key forms

diffuse

Raptor develops even after 3-6 months in the manifest of many syndromes. With a stretch of 1 rock, a great one is taken, a generalized cutting of small curves of the upper and lower kintzivok, Exposing, tuluba. Raynaud's syndrome develops in one hour. Early wines shlunkovo-intestinal tract, M'yazi heart. When videocapilleroscopy of the nail bed appears, the desolation (reduction) of the other vessels begins to rotate due to the establishment of the wormless dylanoks (avascular zones) of the nail bed. In blood analyzes, antibodies appear to the enzyme (topoisomerase 1), which is infused into the uninterrupted DNA molecule.

limitovanu

Harakterizuєtsya Mensch poshirenimi іndurativny shkіrnimi zmіnami, pіznіm i bіlsh povіlnim rozvitkom patologії, trivalim perіodom nayavnostі tіlki Raynaud syndrome, pіznіm rozvitkom gіpertenzії in legenevіy arterії, obmezhennyam urazhennya shkіrnih pokrivіv individual zones, hands i feet, pіznіm rozvitkom calcification shkіri, teleangіoektazіy i porazki herbal tract... During the capillaryoscopy, the expansion of the other vessels begins without the manifestation of rotational avascular zones. In the analyzes of venous blood, specific anticentromeric (antinuclear) autoantitilaprotizing components of the nucleus of the clitin appear.

perehresh

Typical for the general form is the recurrence of symptoms of systemic scleroderma with symptoms of one or the other systemic pathologies of the associated tissue - with rheumatoid arthritis, with systemic cervical arthritis, with dermatomyositis or polymyositis.

Scleroderma without scleroderma

Anyway, it is a visceral form, which protects without impairment of scleroderma, ale with Raynaud's syndrome and signs of damage to internal organs - with fibrosis of legends, development of severe scleroderma, herbal heart disease. In the blood, autoimmune antibodies up to Scl-70 (nuclear topoisomerase) begin.

Juvenile systemic scleroderma

The cob grows up to 16-ryku for the type of line (often asymmetric) or middle scleroderma. In case of linear - children of children with cicatricial changes (prickle on the scalp, nasal bridge, on the forehead and individuals, growing on the lower edges and chest cells) may have a lingering character. With this form, mental capacity is indicated before the formation of contractures (intercourse of ruffs in the area of ​​slopes) and the possibility of manifestation of anomalies in the development of symptoms. Pathological changes in internal organs do not appear significant or appear, even more importantly, with instrumental preliminaries.

indukovanu

The development of which is clearly tied in with the inflow factors of the mid-season (cold, cold and in.). The expansion of the school is broader, often diffuse in nature, in some cases in the midst of court battles.

Prescleroderma

It is clinically manifested as an isolated Raynaud's syndrome, which results in the characteristic capillary picture and / or immunological symptoms, characteristic of the capillaries.

Variation of systemic scleroderma, depending on the nature of the overrun and rate of progress

1. Gostry, a very progressive option - by stretching the first 2 rocky out to the ear of ailment, generalization of diffuse fibrosis of small curves and internal organs, especially lungs, heart and nirok develops. Earlier, in a large number of cases, the disease quickly ended with a fatal result. With a very adequate therapy, the prognosis is decreasing.
2. Pidgostry, along with progressive. For key symptoms and data from laboratory diagnostics, it is characterized by the signs of an immune firing process - shkіrny shkіrnogo nadrya, myositis, arthritis. Non-lonely vipad є perekhresnі syndromes.
3. Chronic, generally progressive. Tsei option of systemic scleroderma to adapt to: Sudden's battle- trivale (stretching out a bagatech of rock) on the first stages of becoming ill with Raynaud's syndrome, which is supervised by a general development along the lines of change of shkiri; we will act on the build-up of razlad_v, knitted with ishemian (ruinous grub) fabric; we will proceed with the development of the legacy hypertension and the damage to the herbal tract.

stages of becoming ill

1. Pochatkova - the appearance of 1 to 3 localization of the ailment.
2. The stage of generalization, which shows the systemicity of the battle with the polysyndromic nature of the manifestation of the process.
3. Thermal, for example, as it is characterized by a lack of function of one or more organs - dichal, heart or nirkova lack.

Victory of three pererakhovannyh parameters in the formulation of diagnoses of ill health allow the development of a child's development by the programs.

Main symptoms

The mechanism of the development of systemic scleroderma and the broadening of the rate is involved in the mechanism of development of the systemic scleroderma, and there is a large number of symptoms and symptoms of ailments. However, looking at the stage of development of the process, there is a sense of the possibility of diagnosing pathology at the early stages of development, forecasting and pouring into the triviality of life of ailments.

Diagnostics is carried out on the basis of the main characteristic primary and more common signs:

1. Striking the shkіrnykh curvatures at the viglyadі shkіrnichnogo embryo.
2. Sudden failure and Raynaud's syndrome.
3. Defeat the support-arm apparatus.
4. Internal organs changes.

Skargi sick at early stages

Ailments are indicative of weakness, lumpiness, lack of durability, uneasy I will adjust the temperature, I don’t change 38 °, decrease my appetite, masti tila and ін. It is important to show signs of being susceptible to diffuse forms of systemic scleroderma that are not specific and do not allow the appearance of an ear of pathology before the appearance of characteristic symptoms.

Shkіrni pokrivi і mucous shells

The diagnosis of shkіri є one of the main diagnostic symptoms of illness and develop in more ailments for systemic scleroderma. The process of the characteristic changes of the shkiri, which is located importantly in the area of ​​exposure and hands, in its own development, go through the stages:

• shchіlnogo nabryak;
• indurative;
• atrophic.

The stench is produced to create a mimic ("hypomimia"). The person of a sick person swells with a characteristic "mask-like" look - the shkіra of the guilt is shaken, dented і taut, the tip of the nose is stiffened up, near the mouth there are vertical folds and wiggles, picked up like a pouch mouthwash... Systemic scleroderma can be associated with Sjogren's syndrome.

The wrinkles of the hands rotate into sclerodactylia, which is also characterized by the same shielding embryo, fibrosis, and induration of the skin, which can lead to the appearance of scotchiness, especially the increase in the number of bushy bunches, the shape of the palm.

Symptoms allow you to diagnose the diagnosis in the first case of a patient.

In case of diffusional forms of incapacitation, the embryo, induration and atrophy of the skin curvatures go beyond the boundaries of the face and the brushes. The stench will widen on the skirts of the tulub, the lower and the upper ones. The order of these signs is not often promoted by children of schoolchildren with either a diffusely wider decrease in pigmentation or an increase in depigmentation, as well as with an intermediate or diffusional hyperpigmentation.

From shkіroyu, as it is more noticeable, calcification is formed (the purchase of calcium salts), which can lead to syrupy necrosis, ruining fabrics and making changes to the appearance of curdled mass (in)

For the establishment of an early diagnosis, a 4-point technique is used, the "shkirny rakhunku" method, which allows assessment of such early manifestations, such as the cob steps, the shkiri for rakhunok and nabryaku. The method is based on palpation of children in 17 weeks - in the area of ​​disguise, breast cells, abdomen and symmetrical patterns of the upper and lower figures. The results will be evaluated in balls:

• visibility of any changes - 0 points;
• The range of curvatures is insignificant, as it is relatively easy for the curtain, but more important, it’s not too slinky, you can add it to the fold - 1 point;
• the schilnist is reconciled, if the school is forced to go into the fold - 2 bali;
• swivel, "board-like" - 3 bali.

In case of a preschool biopsy, an intensive fibrosis is indicated.

How can systemic scleroderma of viclikati post-life undead?

Slimy shells urazhayutsya to finish often one hour in shkіroy. It does not manifest itself with subatrophic or atrophic rhinitis, so that it is important to suffer from the development of permanent dryness and nasal nasal congestion, pharyngitis, stomatitis, abnormalities in children, atrophy and shortening characteristic familiar leaching in the procession shells.

Sudden pathology

Often go to the shkіrnyi destructed. Vaughn is an early and frequent manifestation of systemic scleroderma, as a generalization (enlargement) of the nature of the ailment. Naihakternіshoyu familiar with forehead pathology є Raynaud's syndrome. Win is a symmetrical spastic crisis of the arteries and arteries, as a result of which the proper blood in the tissue (ischemia) is destroyed.

Attacks are supervised by a last two-to-three-phase change in color (whiteness - cyanoticity - harshness) of the small crooked fingers of the hands, wider than the toes, with one-hour accidents in them of pain, paresthesia, onim I want the main localization - the whole fingers of the hands, ale the symptoms may tend to expand without the middle to the entire hand, feet, and sometimes to the nose tips, move and podboriddya, wicked disarthritis.

At the zvyazyka z tim, scho spasms vіdbuvayutsya in the vessels with changing stinks, the attack has a podvzheniy character. Attacks to Raynaud's syndrome can cause symptoms of mimicry, and more often the stench develops under the influence of a cold or psychogenic factor.

Їх pivot to be assessed in steps abo balls:

• Stage I - the manifestation of only changes in school without subjective changes and trophies.
• Stage II - the feeling of pain, poking or oniminnya in the fingers before the attack of the syndrome. The appearance of single scars on the nines of the fingers can be seen.
• Stage III - swelling of pain in case of abnormal / unsuccessful one-by-one swings.
• IV stage - many turns or gangrene.

Sudden spasms and changes in their walls to cause damage to tissue and trophic dislocations - development, dryness and damage to the skin, deformation of nigths, in painful, trivial and difficult hours of recurrent

Trophical wiggles grow, more importantly, on the fingernose phalanges of the fingers ("digital wiggles"), as well as in the smiths of the best mechanical infusion - in the area of ​​small and small loams, buttocks. On the distal phalanges of the fingers, dotted scars (a symptom of a "squishy bite") do not appear often, which were formed as a result of atrophic processes.

The fingers of the fingers change in volume, groaning behind the swelling of the cysts of the nigth phalanges (acroosteolis). In addition, skin necrosis and gangrene can develop with a further self-amputation in the distal and middle phalanges.

With a chronic interruption of the process on the face, anterior and posterior surfaces of the breast tissue, on the lips, on the mucous membranes of the lips, hard children, on the move it is not possible to see the teleangiectasis, through the worms with manifestations of systemic scleroderma.

Support-rucho system

Efficiency of loams and navkoloslobovyh fabrics

Most frequent, and in some and the first manifestations of systemic scleroderma є reduction of slopes, which are manifested:

• a symptom of "rubbing a tendon", which is often overwhelmed by shkiri; win winyak as a result of sclerosis of the tissue of the tendon and the tendons themselves; active members they have;
• polyarthralgias, before polyarthritis behind the type of rheumatoid, ale without swelling destructive snakes in the slopes; at the same hour, erozіynі snakes of the lumpy surfaces appear in 20% of the ailments;
• skut_styu in the slopes, especially the hands, especially when sleeping;
• the development of zginal contractures in the slopes, pumovlenі, in the main, snakes in the synovial membranes, navkoloslobovyh ligaments, tendons and myases;
• osteolysis (deformity) of cysts in the zone of distal joints of the finger phalanges, which deforms and shortens the bones, as well as osteolosis of the lower third of the distal distal exchange trays.

Earbud of arthritis from the most powerfully transverse forms of systemic scleroderma and undergoes current.

Mingling of meazo fabric

To turn around one of the forms of myopathy (myazovaya dystrophy):

• non-progressive fibrous myopathy of non-fatigue nature - the most common form with a wide range of illnesses; to be manifested by a small amount of muscular weakness in the groups of mussels of the proximal growth and insignificant changes in the amount of creatine phosphokinase in the blood (an enzyme that can be found in the tissues of the meat);
• fiery, fiery sufferers are weak and have pain in the blood, 2 times more creatine phosphokinase, and also fiery changes in the results of premonitory biopsy and in the results of electromographs.

In addition, the diffuse form of debility is supernatural, it is the development of myazovy atrophy, vicious contractures and the collapsed loamy crumbling.

Internal organi

Shlunkovo-intestinal tract (gastrointestinal tract)

Systemic scleroderma with lesions of the shlunkovo-intestinal tract occurs in the middle of 70% of ailments. You can wonder whether you see the herbal tract, or in 70-85% - the whole stravohid (scleroderma esophagitis) and intestines.

stravohid

Hypotonia (decrease in tone) to the stravohode is the most frequent form of damage not only to the rest, but to the whole shlunkovo-intestinal tract. The morphological basis is the fibrosis, and the atrophy of the smooth muscles of the stinoks is expanded. characteristic symptoms- it is difficult when kovtanni, post-stove, seeing the grubby breast close behind the sternum, to be able to accept the reception in a horizontal position.

When provedennі ezofagogastroskopіі i rentgenologіchnogo doslіdzhennya viznachayutsya zvuzhenі nizhnі vіddіli stravohodu through scho priyah tverdoї i suhoї їzhі uniquely uskladneny, i rozshirenі verhnі (2/3) vіddіli, vіdsutnіst Hvilya peristalsis i vіdsutnіst elastichnostі stіnok (rigіdnіst) іnodі mozhliva nayavnіst grizhі stravohіdnogo open to dіafragmi ... As a result of the low tone of the HSS, there is an injection of acidic shlunks instead of into stravohid (gastroesophageal reflux) and the formation of new erosions, cicatricial inflammation, superficial breast pain in the liver.

With a trivial overload of GERH, in some ailments, it is possible to replace the stratochid dysfunction of the mucous membrane with cells that are identical to the dysfunction of the mucous membranes of the shlunk or to induce the small intestine (metaplase).

Slunker і twelve-fingered intestine

Hypotonia of the slunker and twelve-fingered intestines є the reason for the destruction of the evacuation of the grub and the closure of the slurry. Tse wiklikє feeling the shrewd weasel for an hour, frequent haircuts More and more cases of seriousness in the epicranial dilences, and some of the slunk bleeding through the formation in the mucous membranes of multiple teleangioectasis, erosions and diseases.

Snakes in the intestines

There is a significant increase, in the case of a stravohode, behind the blame of the intestine, the frequency of the decrease is also the same. However, the symptomatology of intestinal pathology in all types of systemic scleroderma often becomes provincial. Naybilsha characteristic є:

• Signs to duodenitis, how to fumble with a twisted ailment;
• with the development of pathology in small intestine collapse of wetness, as well as abdominal swelling, symptoms of partial paralytic small bowel imperfections (small), malabsorption syndrome - carry parts with a great amount of fat instead of fat in the feces (steatorrhea), to be guilty of constipation and to produce a significant decrease in fat;
• with lesions of the intestinal tract, there is a feeling of laxity and part of constipation (less than 2 independent acts of defecation per day), non-limiting feces, a strong development of frequent recurrent intestinal problems.

Organi dihannya

More than 70% of cases are observed and in the last ten years they became the main cause of death in the middle of ailments for systemic scleroderma. Uterine lesions are susceptible to recurrent perifocal pneumonia, formations of emphysema, papleural cysts, abscesses, pleurisy, recurrent spontaneous pneumothorax, cancer of lesions, which develop in 3-5 to 10 ) development of a legacy lack. The snakes in the legends oppose two clinical and morphological options in the viglyad:

1. For the interstitial type of lesions (interstitial incapacitation of the legends), it is characterized by legeneous fibrosis and diffuse pneumosclerosis, which are maximal in the lower portions of the lungs. Pathological changes develop in the same stretching of the first five rocks of ailment and the greatest turn in people with a diffuse form of ailment. Clinical symptomatology of systemic scleroderma does not appear to be specific - dry cough, irregularly haunted, sluggishness with obstructive vividness, swelling of urticaria and the appearance of crepitant wheezing, so that a “celibate”
   When there is a decrease in the life of the lungs, the increase and deformations of the lungs in the lower parts (on the roentgenogram), with a computer tomography - an unreasonable darkening of the tissue of the lungs (symptomatic) of the dull ...
2. Isolated (primary) legacy hypertension, as a result of leg lesions, or secondary (in 10%), which develops in cases of interstitial pathology in different stages systemic scleroderma. Legendary hypertension of both types often develops after 10 years from the ear of ailment in 10-40%. The head symptom is a very progressive (with a stretch of decilkokh misyatsiv) bad. The main acceleration of hypertension - heart of legend from right-handed lack, as well as thrombosis of the leg artery, which is fatal, as a rule, the result.

Snakes in the heart

I am one of the most uncomfortable and frequent (16-90%) localization of illnesses and to be on the first place among the reasons raptor deaths ailments from systemic scleroderma. Zm_ni polagayut at:

• wreckage of power and wreckage heart rhythm(In 70%), especially to lose the forecast of becoming ill;
• development of myocarditis (in the whole range of vizhyvannya the most low), especially in the middle with polymyositis;
• urazhenn_ internal heart shell (endocardium) with the development of valve vads, over a two-stalk valve;
• the development of an adhesive abo (rіdshe) exudative pericarditis, normal viclicati tamponade of the heart;
• heart failure, as it develops even more rarely, or is characterized by stiffness to the consumption of correcting drugs.

The main symptoms are a lull in case of minor physical problems or in a calm state, in view of discomfort and dull pains in the sternum and malignant pains in the sternum and malignant pains in the area of ​​the sternum;

nirok battle

Craftsmen of the presence of the occasional effective drugs, Acceptably grow. In їkh basis lie the changes of arteriol nirok, which are the cause of intercourse necrosis of nirk tissue, in addition to damage of adequate blood posture.

Most often, changes occur latently, with insignificant functional impairments, amalgamation of deprivation with analyzes of blood and blood. Glomerulonephritis develops latently against chronic nephropathy.

The incidence of changes in the appearance of a scleroderma nirkovy crisis (gross nephropathy) develops in the middle of 5-10% (mainly in the case of diffuse SSD). Wine is characterized by a raptus ear and high progressive nirkovy arterial hypertension, the growth of a great bile in the battle and a nirk deficiency. Only 23% of the ailments with the nephropathic hospital see on 5 rocky. At the same time, when the level of damage is more than 15 rocky, only 13% are seen, at that hour, without a lot of acceleration, it is close to 72%.

New methods of diagnostics of systemic scleroderma

We are admittedly new laboratory reports The methods for determining antinuclear antibodies (ANA) are used:

• antibodies to topoisomerase-1 (Scl-70), as in the presence of isolated Raynaud's syndrome, the development of systemic scleroderma (often diffusional);
• immunogenetic markers HLA-DR3 / DRw52; The presence of antibodies up to Scl-70 in the test results in a 17-fold increase in the risk of pulmonary fibrosis;
• anticentromeric antibodies - present in 20% of ailments, as a rule, with a limited form of pathology; it is also used as a marker of illness in the presence of isolated Raynaud's syndrome;
• anti-lymphocytes to polymerase III RNA - occur in 20-25%, which is important, in case of diffuse form and severe niroc; stink of association with unfriendly forecast.

On the other hand, the appearance of some autoantitils begins, the frequency of the appearance of these autoantibodies is significantly lower. Antibodies up to Pm-Scl (3-5%), up to U 3 -RNP (7%), up to U 1 -RNP (6%) and deyaki іnshі are introduced to them.

Clinical recommendations for systemic scleroderma, proponated by the organization "Association of rheumatology in Russia", include additional instrumental methods of monitoring, allowing the organization to clarify the nature of the general problems

• for the herbal tract - esophagogastroduodenoscopy, contrast radiography, pressure gauge in the stravohode, endoscopic shlunkov pH-meter, biopsy of the metaplastic stravohode;
• for the dichotomous system - bodypletismography, computer tomography of the highest permissiveness, visualization of the new state of affairs and the legacy of diffusional health for an additional spirometry and the implementation of the technique of one-time inhalation in the eyes of the mind;
• for the recognition of legacy hypertension and heart rate - doppler-echocardiography, electrocardiography and catheterization of right-sided heart disease, holter-electrocardiographic monitoring, radioisotope scintigraphy;
• for shkiri, myaziv, synovial shell of loam and tissue of internal organs - pre-biopsy;
• wide-field videocapillaryoscopy of the nigty bed, "shkirny rakhunok" (description of the vische).

Differential diagnosis

Diferentsіyovana dіagnostika sistemnoї sklerodermії conducted of such hvorobami i syndromes spoluchnoї tkanini, yak systemically Chervoniy vovchak, dermatomіozit, revmatoїdny arthritis, hvoroba Reynaud obmezhena sklerodermіya, scleredema Bushko, psevdosklerodermіya, multifocal fіbroz, sklerodermіya, asotsіyovana of puhlinoyu, Werner's syndrome i Rotmunda - Thomson.

Diagnostics of systemic scleroderma is based on the presentation of clinical symptomatology (it can be seen as a change), instrumental and laboratory methods. "The Association of Rheumatologists of Russia", for the same purpose, has recommended the following criteria for differential diagnostics... For the establishment of a reliable diagnosis, the presence of 3 main ones from the lower part is indicated by one of the main ones (scleroderma changes in the shkiri, characteristic changes in the organs of etching, osteolysis of the lower phalanges) in the latter

The main ones are:

1. Scleroderma character of shkirny stripes.
2. Raynaud's Syndrome and Digital Infraction / Abnormal Scar.
3. M'yazovo-saglobovi urazhennya with the development of contractures.
4. Calcification of shkіrnykh curvatures.
5. Osteoliz.
6. Fibrosis of basal lesions.
7. Urazhennya shlunkovo-intestinal tract scleroderma character.
8. Development of large-focal cardiosclerosis with impaired conduction and heart rhythm.
9. Sclerodermic gostra nephropathy.
10. Characteristic results of video capillaryoscopy of the nail bed.
11. Detection of such specific antinuclear antibodies, yak, it is important, up to Scl-70, anticentromeric antibodies and antibodies to polymerase III RNA.

Additional signs:

• The consumption of oil is more than 10 kg.
• Damage to the trophism of fabric
• The appearance of polyserosite, as a rule, an adhesive (adhesive) form.
• Teleangiectasia.
• Chronic interruption of nephropathy.
• Polyartralgia.
• Neuralgia of the tricular nerve (trigmenitis), polyneuritis.
• Improved SHOE indicators over 20 mm / year.
• Gamma globulins appear in the blood, which is 23%.
• Detection of antinuclear factors (ANF) or autoantitils to DNA.
• Revealing of rheumatoid factor.

Treatment of systemic scleroderma

Lіkuvannya zhvoryuvannya is trivial, as a rule, more. It is guilty that it is carried out in a comprehensive manner, in terms of the occurrence of pathology, the nature of the flow and occurrence in the process of quiet organs and systems.

The effectiveness of therapy is significantly reduced by the presence of various factors in risk, as well as the manifestation of such provocative officials, such as improper eating, currying (!), Living alcoholic and energetic (!) lack of help.

How can you be smagaty with systemic scleroderma?

Ultraviolet viprominuvannya є one of the highest factors in the risk of infection, which can make the disease worse. To that, perebuvannya in unprotected sleepy exchanges in mice, especially in the period of stronger sleepy activity, it is not preferable. There are no indications on the Ukrainian sea coast, but only in the autumn months for the reason for the transfer to the city. It is also necessary to start using the cream at the maximum level for the treatment of ultraviolet exchanges.

specialties of food

Significant ma kharchuvannya in case of systemic scleroderma, which is guilty of being bagatorazym with short interruptions between priyomas and in small expenses, especially in case of severe strain. We recommend the inclusion of allergenic herbs and the implantation of products with ample amounts of food (milk and fermented milk products, not sour milk, meat and ribs), micro-macroelements, especially calcium salts.

In case of the deterioration of the function of nirok (nephropathy, nirkova deficiency), the incidence of problems is strictly dosed, and in case of deterioration of the diseases in the herbal tract, it is responsible for the loss of organisms and the processing of the food, the specific

Bazhanimi also є the introduction of carbohydrates, especially when taking glucocorticosteroids, and a sufficient number of vegetables, berries and fruits with a small amount of sugar.

Principles of drug therapy and rehabilitation

The main goals of therapy are:

• reaching the stage of remission for the maximum possible suppression of activity to the process;
• stabilization of the functional camp;
• prophylaxis of acceleration, tied due to wines in vessels and progressive fibrosis;
• preventive maintenance of internal organs or correction of any obvious damage to their functions.

Particularly active therapy is guilty of being in the first place of death for the development of illness, if the main and most significant changes in the systems and organs of the body are intensively observed. You can still reduce the pitch of the turn ignition processesі change the inheritance of viglyadі fіbroznyh wines. Moreover, there is still a possibility of inserting on the already formed fibrous changes in the plan of a private turnaround development.

1. Kuprenil (D-penicylamine) in tablets, which is a volodya antiapaline drug, injected into the metabolic processes in the resulting tissues and in the form of an antiphlogistic effect. Remaining realizable only by holding it out with a stretch of pivrok - rock. Kuprenil is a Vibor preparation in case of severe progressive pathology, diffuse school-related inductive processes and active fibrosis. Wine is recognized in incremental increases, and then doses decrease. Advantageous doses can be taken from 2 to 5 rockets. In connection with possible side effects (toxic effect on nirki, deterioration of intestinal function, dermatitis, infusion on blood-forming organisms, etc.)
2. Immunosuppressive Methotrexate, Azatioprin, Cyclophosphamide і іnshі. Methotrexate volodya is an effective way of suspected shkirny syndrome, with lesions and lobes, especially at the early, heat stage of ailments. Cyclophosphamide is stagnant with high activity process, interstitial lesions with the formation of a lung fibrosis (absolutely not indicated for stagnation), the manifestation of evolving immunological disorders and in the type of disease
3. Enzyme zasobi (Lidase and Ronidase) - degrades mucopolisaccharide and reduce viscosity hyaluronic acid... It is used in chronic processes by courses of pediatric or internal diseases, as well as in vigorous ionophoresis and aplication in the area of ​​tissue induction or contractures.
4. Glucocorticosteroids (Dexamethasone, Metipred, Prednizolone, Triamcinolone) - are recognized during the activity of process II or III stage, And also in vipadkah gosudarny abo pidgostrogo interrupts. Їх zasosuvannya zdіysnyuєtsya with continuous control of the function of nirok.
5. Sudinnі zasobi - Basic Je blockers kaltsієvih kanaltsіv (Corinfar, Nifedipine, Kordafleks, Foridon) іngіbіtori angіotenzinperetvoryuyuchogo enzyme (captopril, Capoten i іn.) SSMSC priznachayutsya Vzhe on Pochatkova Etap hvorobi, prostanoїdi (Іloprost, Vazaprostan) antagonіsti endotelіnovih retseptorіv (Traklіr , bosentan).
6. Antiagreganti (Curantil, Trental) and anticoagulants (small doses acetylsalicylic acid, Fraxiparine).
7. Non-steroidal anti-fumes (Ibuprofen, Nurofen, Piroxicam, Indomethacin) and aminochinolinov (Plaquenil) kosti.

A new method є stasis for systemic scleroderma of genetic engineering biopreparations. In Denmark, the hour is a triumph of vivchennya їkh efficiency and perspectives of stasis for important forms of systemic scleroderma. The stench is a remarkably new straightforward in therapy and other systemic illnesses of the resulting fabric.

Before Tsikh zasobіv vіdnosyatsya Etarnetsept i Іnflіksіkamb scho prignіchuyut autoіmunnі reaktsії, іmunodepresanti Rituximab scho predstavlyaє themselves monoklonalnі antitіla to B-lіmfotsitarnim retseptorіv (in poєdnannі of nizkimi doses glyukokortikoїdіv) antitіla to transformuyuchy growth factor beta-I, antіmonotsіtarny іmunoglobulіn, cytostatic Іmatinіb, it brings over-excess synthesis of microcrystalline matrix, as a result of which there is a change in the syndrome of the syndrome and the function of the legends in the diffuse form of SSD, gamma and alpha-interferoni.

Likuvannya with folk medicine

The complex of lіkuvannya bazhano includes і vasobіv of folk medicine. However, it is necessary to remember about those who are not guilty of using systemic scleroderma by folk methods, but they are not guilty of being one or only in the main one. You can only serve other rows of supplements (!) Before the main therapy, designated by the Fakhivtsy.

With this you can vikoristovuvat roselinna oils, as well as real Lykarsky Roslin(Zvіrobіy, calendula) on rose oil, which is necessary to make a dough on the day of the fight for the day of the fight for the children to help them, to improve the grasping and change the steps in the heating process. It is corny for loams, shkiri and sudin, take warm baths with infusions of geraniums, rhubarb, pine brunoks or goloks, birch leaves, straws.

Protistopalny and immunosuppressive powers volodyut alcohol tinctures or infusions (for the reception in the middle) Saponaria likarskoy, buckwheat Sakhalin, tea root harpagophytum, infusion of herbs from horsetail, honey jelly. Protistopalny and sudinorezdiluvalnuyu effects of Volodya Nastya from such sumy of roslin: immortelle, animal, burkun likarsky, meadow geranium, meadow stables, trees, bird knotweed, Leaves of meat, plantain and mother, berries of raspberries and cranberries, root of kulbabi. Іnuyut і rich lіkarskih lіkarskih roselin near viglyadі zborіv.

Massage i right, physiotherapy

system complex therapy reabilitation also includes (in case of daily activity or insignificant activity): massage and the complex is right in case of systemic scleroderma, to improve the function of dysfunction and heart, regulate the tone of the blisters, polyphenation of crushed skin. the course of physiotherapy - electrophoresis with anti-pyretic, magical and enzyme preparations (Lidaza), thermal procedures (paraffin, ozokerite), aplication with dimethyl sulfoxide for the best loss of loam; sanatorium and spa resort (mud bath and balneotherapy).

Is there a chance to have a visit and a chance to win a child?

The vitality of supervising is to consume significant hormonal changes in the body, with a high rizik for a woman in the plan, she will be overwhelmed, and also a rizik for a fetus and a child. Tim is not mensch, it is possible. Systemic scleroderma is not an absolute contraindication for vaginosity and bedding in a natural way. Especially a high chance of blaming the child for cob stages becoming ill with permissiveness or chronic interruption during daily activity, the process and abnormal changes from the side of internal organs, especially nirok and heart.

However planuvannya vagіtnostі Got Booty obov'yazkovo uzgodzhene of lіkuyuchim fahіvtsem virіshennya to supply about mozhlivіst skasuvannya Pevnyi lіkarskih preparatіv i korektsії lіkuvannya in tsіlomu іz zastosuvannyam hormonal tsitostatichnih, sudinnih, antiplatelet agents zasobіv, preparatіv scho spriyayut polіpshennyu tkaninnogo metabolіzmu i t. D. Krіm In addition, during the period of vaginosity, it is necessary to be prevented from taking care of it not earlier than once in a trimester, not only from an obstetrician-gynecologist, or from a rheumatologist.

In the first trimester, the woman was hospitalized in the hospital in the first trimester, and in the later period, when she was suspected of activating ailments, or for an accelerated change in her health.

Zdіysnennya svoєchasnogo adequate lіkuvannya, right pratsevlashtuvannya, dotrimannya ailing rules postіynogo dispensary sposterezhennya, usunennya abo Institution to mіnіmumu provokuyuchih chinnikіv, vplivu chinnikіv riziku dozvolyayut upovіlniti progresuvannya zahvoryuvannya, uniquely zniziti stupіn agresivnostі Yogo perebіgu, polіpshiti forecast vizhivannya that pіdvischiti yakіst Zhittya.

Chronic recurrent aphthous stomatitis is carried to widespread sickness of the mucous membrane of the empty company and is characterized by the development of ailing recurrent single or multiple changes in the mucous membrane of the empty company. The enchantment was first described in 1884 by Miculicz Kummel, and later in 1888 by Ya.I. Trusevich.

Chronic recurrent aphthous stomatitis (HRAS):

HRAS, fibrinous form. The third day of the ceremony.

Ethiology of chronic recurrent aphthous stomatitis

bacterial infection(L-form α-hemolytic streptococcus Streptococcus Sangvis)

The whole microorganism is always seen from the elements of the fight in patients with typical aphthous disorders. Yogo introduction to the experimental creatures of the Wiklikє of the appearance of the elements of the exercise. It is meant to increase the sensitivity to the introduction of streptococcal antigen.

autoimmune reactions

Look at the manifestation of an autoimmune reaction of the oral epithelium. However, the normal level of antinuclear antibodies and complement does not allow the HRAS to be seen as an autoimmune disease, due to the central immune mechanisms. With HRAS, the local immune response to the antigenic change in the mucus membrane of the empty rota.

Friendly factors:

Colitis ulcers

Crohn's ailment

Reiter's syndrome

cyclical neutropenia

megaloblastic anemia

Fatal deficiency anemia

T-immunodeficiency

local injury

hormonal changes

psychogenic officials

allergic reactions

Pathogenesis of chronic recurrent aphthous stomatitis

L-form of α-hemolytic streptococcus Streptococcus Sangvis іnfіkuє epіthelіy ducts of other slimy salmon, leading to the development of chronic inflammation. With the proliferation of microorganisms, an overwhelming number of antigens accumulates and humoral immunity is stimulated. The antigen-antigen complex, which precipitates on the walls of the sudin, activates the complement system, blood loss, and induces thrombosis, ischemia and necrosis (reaction of Artyus is an immune complex type of immune deficiency , which can expand with the blood flow, leading to the emergence of vasculature and deterioration of other organs and systems).

The process will accelerate the appearance of autoimmune reactions to occur as a result of tissue antigen necrosis. Confirmed autoantitila are glued with epithelial cells of the spiny ball and stimulate autoimmunocomplex damage.

Histology of the fibrinous form of HRAS

Negliboka virazka, covered with fibrinous nalot. Intense neutrophil infiltration in the lamellar mucous membrane under the zone of superficial necrosis. Mononuclear cells, even lymphocytes, are more likely to dominate. In the production of the size, the growth of granulation fabric is indicated.

dribnі salivating with manifestations of per_alveolar and per_tubular fibrosis, chronically fired, Expansion of the duct of silny vines. (Gosstroi zapalennya beforeє chronіchno zapalennya. Such changes of thin worms are indicated when there are flights). Post-production of the ducts of other silty trees.

Element of training with HRAS є abo eroziya, abo twist. The superficial erosion is a defect in the rounded shape, with a size of 2 to 10 mm, covered with fibrinous nalot;

Classification of HRAS

The most sophisticated classification of HRAS. See great and small forms of HRAS; by severity - light, medium and important form.

I.M. Rabinovich (1998) vidіlyaє nastenі form:

fibrinous

necrotic

glandular

deforming

In a short period of time there is a lack of classification є the vision of not independent forms, as it is clinically not seen in one form of one.

Fibrinous form of HRAS (aphtha Mikulichi);

Necrotic periadenitis (Setton's aphthae) (recurrent cicatrices of gliboki aphty, deforming aphtha, povzut aphty);

Herpetiformia aphthous stomatitis;

Symptom with Behcet's ailment.

Fibrinous form of HRAS

Most often at women.

- 10-30 rock_v.

recurrence rate- from 1-2 attacks on the rik, until some recurrences lasting a month, right up to permanent interruption.

peredv_sniki

key overrun- one by one or many times (afty), very painful. Appear can overwhelm vuzliki, igniting other slimy vines.

Number of items- from 1 to 100. In most cases 1-6 elements.

Rosemir- from 2-3 mm to 1 cm.

localization- a slimy shell of an empty company, including a large ball flat non-keratinized epithelium.

Techia- it will last for 7-14 days. Fire up to see the statements of the lower scar, or without a visible scar.

afta Setton

Most often at women.

On the ear of the first attack- 10-30 rock_v. If you get sick, you can fix it as it goes around, but more often in front of you there is a fibrinous form of HRAS.

recurrence rate- permanently; Nemay period, if in the mouth you do not want to go one way.

peredv_sniki- more often paresthesia of the mucous membrane, some subfebrile temperature, localized lymphadenopathy, mucous membrane, part of mov.

key overrun- Hvilepodіbniy, trivialy perebіg, to bring to the sutta deformation of the mucous membrane.

Number of items- from 2 to 10, slightly more. Pozucha, the twist is characterized by lagging on one pole, with growth on the other.

Rosemir- from 1 cm to the striking of the significant mucous membranes.

localization- a mucous membrane, covered with a flat spherical non-keratinizing epithelium, however, when growing, it can be widened on the zone with keratinizing epithelium.

Techia- until the beginning of the month. Fire up to see the statements of the deformable scar.

Herpetiform form HRAS

Most often at women.

On the ear of the first attack- 10-30 rock_v.

recurrence rate- the level of success can be measured gradually over the course of 1-3 rockets with remarkably short steps.

key overrun- a lot of other non-glib incidents (afty), very painful. To fix up as small erosions (1-2 mm), as it is possible to grow up and get angry at the statements of the great erosive surfaces.

localization- the elements of the battle can be roztashovuvatisya on whether the company is empty.

Behcet's ailment

To lie at the base of the disease systemic lesion of sudin - vasculitis.

Head symptoms:

Aphthous stomatitis;

Separation of gen_tal_y;

Eye damage (fear of fear, iritis, conjunctivitis, hypopyon)

Very often, the bottom is much more frequent, it is not diagnosed.

other symptoms

Shkirni urazhennya (pioderma, pustulosis vysipannya, papulosis vysipannya, vuzluvata eritema, multiformal eritema);

Artalgii, monoarthritis of great slopes;

Exercise of the central nervous system;

Exercise nirok;

Picking up the CCC.

other symptoms However, it may be of the highest importance for the forecast, however, due to the specificity of the day, the diagnosis may be of a different order of magnitude

Laboratory diagnostics- hypergamaglobulinemia, increased SHOE, leukocytosis, eosinophilia.

Differential diagnosis of HRAS

Differential diagnosis of fibrinous form of HRAS

3 traumatic erosions(Evidence of a traumatic factor, irregularity of erosion, insignificant soreness);

With the second syphilis(Papules grow out on any kind of CODs, including keratinizing epithelium, painless, may be infiltrated, when scraping it is easy to know about the statements of meat-worms of the disease, regret ).

Herpetic stomatitis(Supports gingivitis, defeated red lips); fend for overly slimy, covered with keratinizing epithelium, the first element of combat is chubby, with herpetiform growths, tendencies to preoccupy

Z bagatoformnaya eritema(Polymorphism visipan, zagalnaya intoxication)

Differential diagnosis of Setton's aft:

Vincent necrotic stomatitis of Vincent(Crater-like bends, in some bright necrotic nalot, the beetles bleed heavily, stinking odor, vinikє on aphids, in the middle of which there are spongers).

With mucous-synechial bullosa dermatitis of Lorta-Jacob(The first element is michur, the second one is erosion, during the day of the infiltration, often є the struggle of the eyes).

3 traumatic virazkoy

With cancer virazkoy

With specific paths

lykuvannya HRAS

Mistseve likuvannya:

Usunennya traumatic officials;

Rinse with tetracycline (250 mg per 5 ml of water 4 times a day for 5-7 days);

Applikatsii corticosteroids and antibiotics;

Zneboluyuchi behind the show.

In case of glide paths - the accumulation of proteolytic enzymes.

Home of lіkuvannya:

antibiotics in the middle

tetracycline

Rifampicin (2 caps. 2 r / s)

Tarivid (1 tab. 2 p / s 20 days)

Sodium thiosulfate (10 ml 30% solution i.v. 1 r / d or 1.5-3 g vseredin)

Prodigiosan (according to the scheme, repaired with 15 mcg 1 time in 5 days, a higher dose of up to 100 mcg).

Pyrogenal behind the scheme

Levamizole (50mg x 3 r / s 2 days drink per day or 150 mg once)

Delagil (1 tab. 1 p / d)

Colchicine (1 tabl × 2 r / d 2 ms.)

Aevit (1 ml 1 r / d i / m 20 days)

Histaglobulin (2.0 ml sc once every 3 days)

01.10.2017

Systemic scleroderma (SSc) is a systemic disease of the resulting tissue, which is characterized by fibrosis, hypertension and immunological diseases. step by step reception of internal organs. Unimportant to those often, SSDs are clinically subdivided into two stages after the level of protection of the school - diffusion and intercourse (limitation), the Raynaud phenomenon and its acceleration and universal signs of getting overwhelmed by 95%. Good see, angioopathy in SSD is a vicious microcirculation of microcirculation from ischemic organs, activation of fibroblasts and a further development of the great fibrosis. Reynaud's Phenomenon Potentially not uncomfortable symptom, Oskilki win often progress up to overshoot (in 50% of patients), leading to gangrene of children.

The seriousness of the situation is associated with the formation of structural damage and functional herbal anomalies in case of Raynaud's phenomenon in the framework of the SJS on the basis of the primary (idiopathic) forms of this phenomenon, if there is no evidence of damage to the tissue. Thus, digital vasculopathy is one of the factors leading to chronic ischemic pain and disability in patients with SS.

Primordial Raynaud's phenomenon є clockwork werewolves Vasospastic a phenomenon. Raynaud's phenomenon - an episode of short-term ischemia in cases of vasospasm of arteries, precapillar arterioles and cerebral arteriovenous anastomoses in the presence of cold and emotional stress. Most of the time it’s hard to catch fingers and nig, knicks vuh, nis and nipples. As a rule, snakes to the color of the shkiri recognize three phases: cobble, cyanosis and erythema as a result of compensatory vasodilatation. Klinichnі manifest the phenomenon of Raynaud can be zagrupuvati with an offensive rank:

• most often the changes of the barn are spared on the fingers of the hands;
• Zones can be repaired on one finger, widen on one finger, and symmetrical on both hands;
• the most frequent are II-IV fingers of the hands, thumb zvychay to be too late;
• The change of bargaining can be indicated on the other dilyankas - in the shells, Kinchik of the nose, revealing, over the kolins;
• before the hour of Raynaud's attacks, you can see a frequent livedo at the end of the day, as you pass when the vasospasm is complete;
• in the vicinity of vypadkah sposterіgaє
• signifies a part of ailments to skarzhannya on sensory damage (oniminnya, pokolyuvannya, bil) before the hour of the attack.

The breadth of the Raynaud's phenomenon becomes less than 10% in the foreign population. N.A. Flavahan (2015), in a recent look at the emphasis on thermoregulatory mechanisms and the basis for the understanding of Raynaud's phenomenon, the role of arteriovenous anastomoses and increased activity of α2-adrenoceptor blockers in decreased blood circulation.

Raynaud's phenomenon in SJS inherited structural and functional vertebral lesions with abnormal proliferation of distal arteries (digital arteries). Sudinny snakes may be twofold. On one side, the proliferation and fibrosis of antihypertensives, endothelial activity, is indicated for the production of vascular sound mediators and an hourly decrease in the development of shipborne expanding molecules. On the other hand, part of the episode of vasospasm should be produced in the residual bag until the progressive tissue development, the production of strong superoxide radicals and even more pathological changes in the tissues. The pathophysiology of Raynaud's phenomenon is tied with folding mechanisms and transmission between modality and vascular, internal vascular factors and mechanisms of nervous control.

The diagnosis of Raynaud's phenomenon is established in the first place on the presentation of scarg and clinical symptoms, and it becomes reliable with a positive response on three days of nutrition:

1. What is the significance of the unwitting sensitivity of your fingers to the cold?
2. How do you change the color of your fingers in the cold?
3. Why does the stench faint and / or bluish?

With a positive response to all three nutrition, the diagnosis of Raynaud's phenomenon is reliable.

The second Raynaud's phenomenon is often observed with systemic congestion of the full tissue and with the greatest broadening with SJS (up to 95% of cases), as well as with systemic cervonia (close to 40%), dermatomyositis within the framework of antithyroid syndrome, 25% %). Diagnostic criteria of primary and secondary Raynaud's phenomenon are presented in Table 1.

It is accepted respectfully, that the twisting of the fingers (pads) of the fingers є the inherited ischemia, while the twisting of the original surfaces of the fingers is of a "traumatic" character. Until today, we have not had enough proofs of the theory. However, a message from B. Ruaro і spіvavt. (2015), which included 20 patients with STS and finger twisting, demonstrated a significant decrease in blood flow in the moment of finger twisting and fingering. The ischemia of the tissue can also be the basis for the development of osteolysis, especially the nigth phalanges.

The ability to predict the development of digital paths may be of great importance, but it is not very important to allow a group of patients to see, as they require highly direct, professional injections. For the last hour in the course of the decile dull boules, the predictor of the incidences in SSD and the predictive factor were described. In the great prospective prescient (n = 623), for the participation of patients with the SDS, it was established that before the most powerful factors in the development of new digital virazoks in the course of 6 months, the abnormalities in the number of fingers and the manifestation of any critical ischemia. Priority predictors of antibodies to topoisomerase (anti-Scl-70), antibodies to type A receptor endothelin-1 (ET-1), increased blood pressure A systematic survey of PRISMA I. Silva and spivavt. (2015) discussed the factors of development of digital pathways, such as є: type of diffuse reduction in patients with SS, early debut of Raynaud's phenomenon, the presence of antibodies to topoisomerase (anti-Scal-70), abnormalities growth of endothelium sudin (VEGF).

One hour to see, how the manifestation of digital twists and turns is tied with an important interruption of ailments and to bring about an increased mortality. In the large-factor analysis of 3196 patients from the EUSTAR base, the history of digital changes was a reliable prognostic factor of death.

The mechanism of the development of digital pathways in case of SS is explained by decilcoma factors, which include repeated microtraumas, vitality of shkiri, dryness and manifestation of calcification. It is important that 8-12% of cases are found on the basis of calcification of the skin and pediatric cells. However, the trivial ischemia after the Raynaud phenomenon is the most important mechanism. The digital curves grow in size and between the fringes, the obvious bare tissues (knuckle, tendons), calcification of the tissues. Virazki vvazhayutsya gostry up to 3 m / s, chronic - up to 6 m / s. Clinical results and turns lie in the number of factors. The loss of soft tissues and cells is more likely to occur in approximately 30% of patients with SSD and digitally in a circular pattern. In the course of the 7-course monitoring, there was an acceleration of gangrene in patients with swelling in 11% of cases; in case of ineffectiveness, for the duration of the disease, the manifestation of recurrent ischemic attacks of gangrene development, has been diagnosed in 100% of patients with a year; 12% of patients with digital trips received hospitalization and surgical assistance.

Critical is the ischemia of symptoms in case of SSD є urgent mills and non-emergency visits. Oscillations in the development of critical ischemia are non-reversible ischemia (on the basis of Raynaud's phenomenon), then the process can quickly lead to gangrene of the picture and the loss of fingers. For baby 1 (a, b, c), there are photographs of patients with digital twists and turns and developed as a critical ischemia on the SSD.

Announcement of Critical Ischemia of Supervision badly ill, Scho inodі nіtіvіt vimagає zasosuvannya narcotic analgesics. Naymenshі dotsіkі rukh viklikayut bіl. Appear critical ischemia change the wrinkles of the fingers, the style of beating, the color of the blues, the differences between the "blue" and the "blue" zones of the fingers. It is important to keep your hands on the table not to spoil any positive in the flow (well, maybe, more efficiently earlier). Critical ischemia develop in II-IV fingers. It is unimportant for those that at the basis of vasculopathy in SSD lie vaso-occlusive ailment, swelling and dying of reversible potential and helping to overcome the loss of soft tissues and tighten the fingers.

Patient management with Raynaud's phenomenon, with digital changes / necrosis in SS, including nonpharmacological, pharmacological, and surgical treatment (Table 2). Vikoristovuvany non-pharmacological modalities include the unique triggers, anti-inflammatory drugs, including cold contact, emotional stress, and the use of acute sudin, including β-adrenergic antagonists, anti-inflammatory drugs such as cisplatin, vinblastin, targeted tyrosine kinase blockers, in.) and amphetamines. The pinching of the curtain is absolutely necessary for the capturing of the given court's ear with the already sprinkling ischemic fabric.

Vasoactive methods of therapy are central to the pharmacological treatment of Sudden SJS acceleration. E. Hachulla і spіvavt. (2007), as a rule, vasodilatation therapy will significantly overshadow the development of distal pathways (development of distal pathways, RR 0.17; 95% preconditioned interval, DI 0.09-0.32).

Calcium channel blockers (CCBs) are few in the treatment / prophylaxis of digital virazoks, there is a desire for a large number of clinicians in the vicious group of drugs (most often nifedipin) in the treatment of important phenomena. In a randomized sublingual blind, oral nifedipin was administered (30 mg daily for 4 times, and at the same time 60 mg daily for 12 times) and intravenously administered Iloprost for phenomogenesis important. According to the recognized results, the average number of digital trips was changed from 4.3 to 1.4 in 16 cases of treatment with nifedipin. With the stasis of iloprost, the number of digital signs changed from 3.5 to 0.6. Adjustment of hand temperature and microcirculation is indicated only in case of stagnation of iloprost.

Nezvazhayuchi those scho іsnuє dosit stronger is therapeutic obgruntuvannya rolі іngіbіtorіv angіotenzinperetvoryuyuchogo enzyme (ACE) in SS i sudinnih uskladnennyah in yakostі agentіv remodelyuvannya sudin (yak i y patsієntіv of іshemіchnoyu hvoroboyu sericite) in danii hour vіdsutnya dokazova base yak pіdtverdzhuє efektivnіst tsogo vtruchannya. At bagatotsentrovomu podvіynomu slіpomu randomіzovanomu klіnіchnomu viprobuvannі, yaky consisting of 210 patsієntіv lіmіtovanoї MICs abo autoіmunnim Raynaud's phenomenon (s nayavnіstyu spetsifіchnih sklerodermіchnih autoantitіl) trirіchne lіkuvannya kvіnaprilom not asotsіyuvati Zi digit number zmenshennyam novih digіtalnih virazok (OR 0.08; 95% od DІ - 0.23 to 0.06).

Ingibitories of phosphodiesterase type 5 (PDE5) develop degradation (and, moreover, increase the bioavailability) of cyclic guanosine monophosphate (HMP) with further vasodilation. In the metaanalysis of the effectiveness of the therapy of digital tests, which included 31 randomized control tests for the duration of the test, the rate of testing of the inhibitors of phosphodiesterase-5 (on the basis of three randomized classroom tests, n = 85)

In a recent highly centered sublingual blind, randomized controlled patient, 84 patients were included in the yak; Protect the hour of the start of the tsikh virazok (the main point of the end of the day) did not change. Three commercially available FDE5 Ingibitors include Sildenafil, Vardenafil and Tadalafil. Sildenafil and vardenafil may last for a longer short period in the West - close to 4 years, at that time the period when Tadalafil Nabagato started to last longer - lasting 18 years. Doses of oral vasodilating drugs, which are most often used in the therapy of Raynaud's phenomenon and on the second, are presented in Table 3.

Prostanoid є by straining vasodilators, and also inhibit the aggregation of platelets and proliferation of cells of smooth ointments of sudin. Iloprost, contractions in Europe for the treatment of digital virazoks, tied with SJS, is a chemically stable analogue of prostacyclin with a subordinate vasodilator and platelet effects. Iloprost is a synthetic analogue of prostacyclin, viciousness of aggregation and activation of thrombocytes; activates endogenous fibrinolysis, without the anti-fatigue effect, causing adhesion and migration of leukocytes to the ear endothelium, as well as the accumulation of leukocytes in the ischemic tissues.

With intravenous injection of prostanoids, the frequency of side effects and disgusting tolerance of the drug, including systemic hypotension, spoilage, flushing, gut-intestinal problems, is determined to be as high as 92 percent of the percentage

Internal therapy of prostate people should be seen in case of refractory interruption of Raynaud's phenomenon, especially in patients with generalized form of SSD and in cold weather. Most often it is used for internal prostate (3-5 days of treatment with 0.5-2 ng / kg / h in a stretch of 6 hours, repeated courses of skin 4/6/8 in a stretch of 52 types) and epoprostenol.

I also talked about the internal therapy of prostate for the improvement of the start of digital flights and the rapid number of new ones. In bi-centered sublinear, randomized, early intravenous therapy for prostanoids (iprost 0.5-2.0 ng / kg / h for 6 years for 5 days after sleep), the bullet was tied with significantly larger patients.

In important cases, vasculopathies are overrun, with recurrent problems, patients are guilty of repetitive courses of prostanoids; without interruption or extension of the course of internal therapy to be seen in clinically dead-end situations.

It is necessary to note that oral prostanoid preparations (iprost, and also new preparations - beraprost, cyprost, treprostinil) did not demonstrate any kind of polyphenia in registered digital virazoks. The second prostaglandin analogs - alprostadil - tend to become stagnant in the development of Raynaud's phenomenon and digital virazoks.

Prazosin yak antagonist of the α1-adrenergic receptor in two randomized dosages, demonstrating a friendly inflow to overcome the Raynaud's phenomenon. Apparently, prazosin was consumed in a dose of 1 mg 3 times and the dose was improved and the prognosis was taken from placebo and due to fewer failures in the case of new It is a pity that the published tributes about the second flow on the digital curves are not enough.

Miscellaneous nitrates were used for the improvement of local blood flow, protestably, for the reduction of potential side effects Have changed the entusiasm of regular victories.

ET-1 є does not deprive us of vasoconstrictors by straining, but the proliferative effect on cells of smooth mucous membranes and fibroblasts is increased, acting through two receptors (cya and ETB). In general, ETB, appear in the cells of smooth mucous membranes, take vasoconstriction and hyperplasia, at that time as ETB, which is also found on endothelial cells, spriyas vasodilatation.

Bosentan is a subline antagonist of ET-1 receptors; arterial hypertension(PAH) and prevention of recurrent digital infections. Two great sublinear sublinear, randomized controllers who have been tested have demonstrated that bosentan treatment has significantly accelerated the number of new flights. In a randomized sublingual blind placebo-controlled dose of bosentan for 2 times for start-up and prevention of ischemic digital changes in patients with SSS, which included 188 patients with 24 to add) the association with a 30% reduction in the number of new digital paths. Bosentan contractions in Europe for the prevention of digital infections in case of scleroderma, protest Controls for the control of the quality of products in the United States (FDA) for the analysis of painful contractions is not used. Bosentan can be an important method of drug use, it is possible to use it orally and potentially uniquely in order to acquire new digital transmissions.

In patients with incurable, volatile digital virazoks, refractory to therapy with phosphodiesterase-5 and internal infusions of prostanoids, antagonists of ET-1 receptors can be especially brown.

On the current day for PAH testing in Europe, two new ET-1 receptor antagonists have been hardened: matentan and ammbrizentan, which underwent further testing in the test of digital virazoks in SSD.

Calcification of the tissue, how to feel the twist, you can squeeze the surgical treatment, if you come in after the fire was not successful. Digital (dolonnaya) sympathectomy can bring significant corny to patients who have not been accepted to conservative methods of therapy. Insane cousins ​​є those who methodology given Visitors to the surrounding specialized surgical centers.

On babies 2, 3 and 4, there are adapted recommendations on the management of patients with the Raynaud's phenomenon, on a digital basis and on a critical ischemia. Smells represent a step-by-step option for improving therapy in terms of the prevalence of efficiency or ineffectiveness of earlier visits, based on the advanced clinical practice.

In such a rank, SSA-associated vasculopathy is a serious and urgent problem, which significantly accelerates the transition of SSA. At the same time, it’s easy to carry around, inexpensive, affordable therapeutic options for solving the Raynaud's phenomenon, and it’s faster for people to see digital turns to be a direct priority. Victory of a well-known therapeutic approach to optimize the treatment of patients with the Raynaud phenomenon and digitally allow adequate supervision of such patients and to prepare for the adoption of new children for forgetting

literature

1. Alekperov R.T. Raynaud's syndrome is a multidisciplinary problem. Almanac of Clinical Medicine. 2014; 35: 94-100.
2. Volkov A.V., Yudkin M.M. Intravenous prostate in the complex therapy of vertebral damage in patients with systemic incapacitation of the resulting tissue. Suchasna rheumatology. 2013; 2: 70-74.
3. Sinyachenko O.V., Egudina E.D., Mikuksts V.Ya. і spіvavt. Angiopathy in systemic scleroderma. Ukrainian rheumatology journal. 2017; 67 (1): 5-11.
4. Blaise S., Roustit M., Carpentier P., et al. The digital thermal hyperemia pattern is associated with the onset of digital ulcerations in systemic sclerosis during 3 years of follow-up. Microvasc Res. 2014; 94: 119-122.
5. Block J.A., Sequeira W. Raynaud's phenomenon. Lancet. 2001; 357 (9273): 2042-2048.
6. Botzoris V., Drosos A.A. Management of Raynaud's phenomenon and digital ulcers in systemic sclerosis. Joint Bone Spine. 2011 rock; 78 (4): 341-346.
7. Cutolo M., Herrick A. L., Distler O., et al. Nailfold videocapillaroscopic and other clinical risk factors for digital ulcers in systemic sclerosis: a multicenter, prospective cohort study. Arthritis Rheumatol. 2016 rock; 68 (10): 2527-2539.
8. Flavahan N.A. A vascular mechanistic approach to understanding Raynaud phenomenon. Nat Rev Rheumatol. 2015; 11: 146-158.
9. Gliddon A.E., Dore C.J., Black C.M., et al. Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. Arthritis Rheum. 2007; 56 (11): 3837-3846.
10. Hachulla E., Clerson P., Launay D., et al. Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study. J Rheumatol. 2007; 34: 2423-2430.
11. Hachulla E., Hatron P.Y., Carpentier P., et al. Efficacy of sildenafil on ischaemic digital ulcer healing in systemic sclerosis: the placebo-controlled SEDUCE study. Ann Rheum Dis. 2016 rock; 75 (6): 1009-1015.
12. Herrick A.L. Management of Raynaud's phenomenon and digital ischemia. Curr Rheumatol Rep. 2013; 15 (1): 303.
13. Herrick A.L. Recent advances in the pathogenesis and management of Raynaud's phenomenon and digital ulcers. Curr Opin Rheumatol. 2016 rock; 28 (6): 577-585.
14. Hughes M., Herrick A.L. Digital ulcers in systemic sclerosis. Rheumatology (Oxford). 2017; 56 (1): 14-25.
15. Hughes M., Ong V. H., Anderson M. E., et al. Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis. Rheumatology, 2015; 54: 2015-2024.
16. Hunzelmann N., Riemekasten G., Becker M. O., et al. The Predict Study: low risk for digital ulcer development in patients with systemic sclerosis with increasing disease duration and lack of topoisomerase-1 antibodies. Br J Dermatol. 2016 rock; 174: 1384-1387.
17. Matucci Cerinic M., Denton C. P., Furst D. E., et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomized, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011 rock; 70 (1): 32-38.
18. Maverakis E., Patel F., Kronenberg D., et al. International Consensus Criteria for the Diagnosis of Raynaud's Phenomenon. J Autoimmun. 2014; 2: 60-65.
19. Meier F. M., Frommer K. W., Dinser R., et al. Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012; 71: 1355-1360.
20. Mihai C., Landewe R., van der Heijde D., et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. ANN Rheum Dis. 2015; 75 (4): 681-686.
21. Nitsche A. Raynaud, digital ulcers and calcinosis in scleroderma. Reumatol Clin. 2012; 8 (5): 270-277.
22. Ruaro B., Sulli A., Smith V., et al. Short-term follow-up of digital ulcers by laser speckle contrast analysis in systemic sclerosis patients. Microvasc Res. 2015; 101: 82-85.
23. Silva I., Almeida J., Vasoncelos C. A PRISMA-driven systematic review for predictive risk factors of digital ulcers in systemic sclerosis patients. Autoimmunity Rev. 2015; 14: 140-152.
24. Silva I., Teixeira A., Oliveira J., et al. Endothelial dysfunction and nailfold videocapillaroscopy pattern as predictors of digital ulcers in systemic sclerosis: a cohort study and review of the literature. Clinic Rev Allerg Immunol. 2015; 49: 240-252.
25. Steen V., Denton C. P., Pope J. E., Matucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford). 2009 rock; 4 Suppl. 3: 19-24.
26. Tingey T., Shu J., Smuczek J., Pope J. Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken). 2013; 65 (9): 1460-1471.
27. Wigley F.M. Clinical practice. Raynaud's phenomenon. N Engl J Med. 2002; 347: 1001-1008.
28. Wigley F. M., Wise R. A., Seibold J. R., et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med. 1994; 120 (3): 199-206.

Fibrillation of the front of the heart (FP) is associated with the risk of death, the disease and other thromboembolic acceleration, heart failure and hospitalization, malfunctioning of the quality of life dysfunction, malignant disease AF on the basis of the transferred Gostr coronary syndrome (GCS) є Expansion of the folding in a clinical situation that requires the correction of anticoagulant and antithrombotic therapy (Kirchhof et al., 2016; Steffel et al., 2018) ....

13.01.2020 Cardiology Rheumatology Myocardial infarction in case of university polyarthritis

Unimportant on Significance The perfect strategy for the treatment of patients from the State Gostra with myocardial infarction (IM) for the last ten years, the whole ailment still needs to be deprived of one of the leading causes of illness in the whole world. More than 80% of cases of IM are severely stenotic coronary arteries (CA) atherosclerosis, and in 5% of cases the cause of fatal IM is non-atherosclerotic CA. However, according to the data of J. Saw et al., In women with IM VICOM ≤50 years with the help of coronary angiography (CG) in 28.8% of patients with no signs of artery disease, in 36.4% - atherosclerotic, in 30.3% - non-atherosclerotic lesions. CA is the same in 4.5% - etiology has not been established. ...

RCHRH (Republican Center for Development of Health Protection of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2013

Progressive systemic sclerosis (M34.0)

Rheumatology

General information

Short description

hardened

protocol of the expert committee

food development, protect health of the Ministry of Health of the Republic of Kazakhstan

value: Systemic scleroderma (SSc) is an autoimmune disease of the resulting tissue, the main clinical signs of a broadened microcirculation, fibrosis of the skin and internal organs.

Classification

Klinichna classification (the most widening of the way, for example: by etiology, by stage, etc.).

Клінічні forms
- Diffuse form. Generalized the striking of the shkiri of the pictures, denouncing that tuluba by stretching out to fate; Raynaud's syndrome appears immediately anyway, when the shkiri is overcome. Early development of visceral pathology (interstitial lesion, lesion of the intestinal tract, myocardium, nirok). The reduction of the capillaries of the nail bed with the shape of the vascular holes (according to the data of the capillary of the nail bed) is significant. Evolution of AT to topoisomerase-1 (Scl-70).
- The form is limited. The trivial period of the isolated Raynaud phenomenon. The striking of the shkіri is surrounded by the area of ​​exposure and the hands / feet. Increase in the development of legacy hypertension, lesion of the intestinal tract, telangiectasis, calcification (CREST syndrome). Vyavlennya anticentromeric AT. Expansion of the capillaries of the nail bed without swelling avascular holes.
- Scleroderma without scleroderma. For scleroderma without scleroderma (sclerodermasinescleroderma) is characterized by: mild damage to the shkiri, Raynaud's phenomenon, signs of pulmonary fibrosis, hostile scleroderma, damage to the heart and shlunkovo-intestinal tract, anti-inflammatory
- Perekhresnі form. For perekhresnyh forms (overlap-syndromes) are characterized by a combination of key signs of SSD and one or more decilkoh systemic illnesses with the resulting tissue.
- Juvenile scleroderma. Ear of ailment up to 16 years. The classification of the shkiri is not very common according to the type of vognischevoy abo linea (hemiform) scleroderma. Sleepiness until contractures are established. Possible anomalies in the development of pictures. Pomіrna visceral pathology (to appear as the head rank with instrumental doslіgennі).
- Prescleroderma. Seeing also the so-called prescleroderma, before it carry the ailments with the isolated Raynaud's phenomenon in addition to the capillary symptoms or immunological disorders characteristic of SSD.
options override

1. Gostre, Shvidkoproprosyuchy stretch is characterized by the development of generalized fibrosis of the shkiri (diffuse form) and internal organs (heart, legends, nirok) in the first 2 rocks from the ear of the disease; earlier not very often ended with death; The therapy is now adequate and the prognosis of the whole category of ailments has been improved.
2. In case of pidgostroma, along with a progressive interruption, it is clinically and laboratory-based to identify the signs of immune inflammation (malignant swelling, arthritis, myositis) of non-overlap syndromes.
3. Chronic, progressively progressive change in the development of mild pathology: on the ear of illness - Raynaud's syndrome with a gradual development of small children (modified form), growth in the early stages of the disease Prognostic visibility of options in case of overturning of the illustrator 5- and 10-fold gostromu become 4 і 0%, with pidgostroma 75 і 61%, and with chronic - 88 and 84% apparently. In the Danish hour at the most early diagnosisі happy therapy prognosis of ailments of the SSD is reduced, ale of the evidence in the debut, the main clinics appear and evolve are taken.

diagnostics

Diagnostic criteria:
For verification of the diagnosis of SSD vicorist criteria of the American Rheumatology Association.
A. "Great" criterion. Proximal scleroderma: symmetric sweating, shrinkage and induration of the fingers and proximally from the palatophalangeal and metatarsophalangeal slopes. Zmini can tinker, denouncing, shiyu, tulub ( chest wall and live).
B. "Mali" criteria.
1. Sclerodactyly: perakhovanі vyshkіrnі vyshkіnі, interlaced with fingers.
2.digital scars - dilyanki zapadannya shkiri on the knuckles of the fingers
for the vrata of the speech of the pillows of the fingers.
3. Bilateral basal legenevium fibrosis: bilateral systemic parts or linear-nodular type, the most widespread in the basal dylyankas legeny with standard radiologic dosage; You can show up behind the type of "stylized lung". Even though they are not guilty, they are tied to the primordial battles of the legend.
The criteria allow for the prevention of ailments with local forms of scleroderma, eosinophilnymfastitis and different types of pseudoscleroderma. The sick person is guilty of either a head criterion or a 2 small criterion. Sensitivity - 97%, specificity - 98%. All the criteria for the appearance of the characteristic and to complete the turn of the SSD, or do not hunt all key forms becoming ill, including the early limitovanu, perehresnu and visceral SSD.

skargi: weakness, stomachache, vagi loss, subfebrile fever and in. Spostering in the debut of ailments (in the main in ailments of a diffuse form) and presenting diagnostic difficulties before the appearance of characteristic small and visceral signs of SSD.

Physical details:
Constitutional symptoms - weakness, stomachache, vaginal effusion, subfebrile fever and іn. Spostering in the debut of ailments (in the main in ailments of a diffuse form) and presenting diagnostics is difficult until the appearance of characteristic school and visceral signs of SSD.
Striking the Sudins:
- Raynaud's phenomenon - a symmetric paroxysmal spasm of the digital arteries, arterioles and arteriovenous shunts, cold induction or hemorrhagic stress, characterized by a lasting change in the formation of palpitations. Vasospasm is often superfluous to the onset of fingers and pain. In the case of patients with ailments on the STS, Raynaud's attacks may cause prolongation of the nature of inheritance of structural changes in sudin and permanently reduced blood flow.
- Telangiectasis - enlarged capillaries and venules with characteristic localization on the fingers of the hands, lobes and individuals, including on the lips, є I know ailments.
- Picking shkiri:
Shkіri education (scleroderma) must be repaired from the fingers of the hands (sclerodactyly). The variability of the absenteeism of the pupils is assessed by palpation for a 4-point system: 0 - inferiority is not; 1 - slightly impaired; 2 - about the loss; 3 - bend the fold For the activation of the education of the children, the school starts, which represents the sum of the ballistic assessments of the changes in the distribution of the children in 17 anatomical areas: on the faces, breasts, abdomens and on the fingers of the symmetrical feet - With SJS, the staging of the shkiri is indicated: nagryak, indura, atrophy.
The risk of ailments grows between ailments and reaches a maximum in the first 3-4 years of ailments. Shkіrniy rakhunok is the main reason for visceral pathology and one of the predictors of unpleasant results of SJS.
· The symptom of the "pouch" is a change in the mouth aperture, the vitrification of the red lips, near which radial folds are formed.
· Digital vyrazki - characteristic signs of SSD (inclusions in the classification criteria), develop on the distal phalanges of the fingers of the hands; They can be very painful, they become torpid to the point of failure and recurrent interruption.
· Vyrazkovі urazhennya shkіri are spared і on dіlyankas, so they are fed by mechanical injections, - over lіktyіvіy і colony slopes, in the area of ​​sprays and nyat.
· Dry gangrene - necrosis of skin and tender tissues of the child can be repaired from the distal phalanges of the fingers and can be widened in the middle phalanx with a further demarcation and self-amputation.
· Hyperpigmentation - it is interlaced with either diffuse, with dilenks of hypo or depigmentation ("sil with pepper").
- digital scars - points of the atrophy of the atrophy of the distal phalanges of the fingers of the hands ("squinting bite").
- Atrophy of hair follicles, sweaty and sebaceous vines, Shkira in the mountains is dry and short, hairy curl.
- Calcification - small changes in the amount of calcium salts added to the calcification, they appear on the fingers of the hands and on the plates, often suffer injuries. Calcinati can grow out of the picture of curdled oil.
- Cutting of mucous membranes, a characteristic feature of STS є draining і shortening of the tongue vortex.
Cutting the slopes and cistok
- Polyarthralgia and rank skutism є frequent manifestation of SS, especially at the early stages of ailment.
- Arthritis is uncharacteristic for SJS, at the same hour, 20% of ailments develop erosive arthropathy.
- Acroosteoliasis - resorption of the endoscopy of the distal phalanges of the hands, as a rule, of the trivial ischemia, manifests itself in shortened and deformity of the fingers. - Have children in vipadkah sposterіgaєt rozmoktuvannya distal view interchangeable cysts and sprouts in the lower slit.
- A symptom of rubbing a tendon - crepitus, which can be palpated in ailments with a diffuse form of SJS with active backward and outward collapses of fingers and hands; є a predictor of falsified diffuse lesion of shkiri.
- Zginalny contractures, especially the folds of the wrists, є a trace of local infringement of the skin with the lesions of the tendon and the sheaths. - It often occurs in ailments with a diffuse form of SSD, when there can be contractures and great slopes of children. Strengthening contractures is associated with the activity and progressive interruption of the disease.
Picking up the music:
- The misery of m'yazіv is manifested in two ways different forms myopathy:
Chi is not ignited, but fibrotic myopathy does not progress - є the more frequent form of mutilation in SS, is characterized by a slight weakness of proximal groups of mucous and minimal changes in the CPK level.
Ignition myopathy - manifested by myalgias, proximal myazoi weakness, significant (in 2 or more times) CPK adjustments, fiery symptoms of EMG and biopsy.
- In case of diffuse form of SS, atrophy of muscular tissues can develop, it is associated with rupture of decay and contractures.
Exercise of the shlunkovo-intestinal tract:
- Hypotonia to the intestinal tract is the most common form of damage to the intestinal tract and the intestinal tract as a whole; manifest itself as dysphagia, feel coma behind the breastbone, with a stiff stove, to accept in a horizontal position.
- The stricture is the sound of the education of the lower third of the stravohode, as a result of which it becomes an ill-humored reception of a firm uzhi. The formation of strictures should be made to a significant decrease in the turn of the oven.
- Erosion and twisting of the stratospin are likely to cause reflux, supravodzhuyutsya twisted liver and pain in the chest.
- Hypotonia of the slunk - in the epigastriya, the shvidko advances in response to the loss of evacuation in the place of the slunk.
- Shlunkov bleeding - rіdkіsne, ale seriozne accelerated, can occur with multiple telangiectasis of the mucous slurry.
- Syndrome of malabsorption - manifested by flatulence, steatorrhea, constipation and diarrhea, the second vagi.
- Іntestіnalnaya pseudo-obstruction - rarely accelerated, manifested by symptomatology of parallіticheskogoіleus.
- Dislocation of the intestine to produce constipation (less than 2 spontaneous defecations per day) and non-limiting feces; to meet with the same frequency, as and hypotonia stravohod.
Exercise Legend:
Lung damage is spontaneous in 70% of patients with SJS and, according to the frequency, only hits the stravohode. The main clinical and morphological types of lesions in SSD are interstitial lesions of lesions (fibrosis of legends) and legeneva hypertension.
- Interstitial ailment (IZL) grows more or less in the first 5 years of ailment and is more pronounced in diffuse form of SSD. Clinical manifestations of IDD are nonspecific and include a sluggishness, dry cough and weakness. The characteristic auscultatory sign is ІЗЛ є bilateral basal crepitations, which is often described as “trisk cellophane”. Risk factors ІЗЛ є: diffuse form of SSD, reduced forced life capacity of legends in the debut of ailments and the appearance of Scl-70 AT. On the progress of the legendary fibrosis, a change in the forced life capacity of the legends and diffusional health should be introduced with a stretch of 6-12 msec in front; widening of the type of matte slope і the picture of the "style" legacy at HRCT; increase in the number of neutrophils and / or eosinophils in the lavage line. The clearest equivalent of the progressive ІЗЛ є the improvement of the back.
Legeneva hypertension viz. legendary artery eptus 25 mm Hg at rest or 30 mm Hg with physical attachments. Legeneva hypertension can be primary (isolated) - in the case of a loss of a woman or a second one - as a result of an increase in the interstitial tissue of legends, developing in the middle in 10% of ailments, especially in the form of a disease the main with a classy acquaintance Legendary hypertension, as it is in case of ІЗЛ, є zadishka, as I tend to progress to a quick progress by stretching out decilkoh miscellaneous. Auscultatory familiarity with legacy hypertension є an emphasis and a split of another tone on the legacy artery and tristular valve, especially clearly at the height of the inhalation. Predictor of legacy hypertension and isolation of changes in diffusional health of CO (<60% от должной величины).
Heart break:
Symptoms of heart damage - discomfort or trivial dull pains in the precardial area, heart disease and arrhythmias, lullness in calmness, or at new pains. Pain in the breasts can cause wicclicks, as well as injuries to the stravohode, or the muscles of the chest wall. In patients with severe heart disease, with SJS, it is asymptomatic and appears at an instrumental condition.
Fibrosis of the myocardium of shlunochkov is a characteristic pathomorphological sign of scleroderma lesions of the heart, є the cause of systolic and dysfunctional dysfunction of the shlunochki due to decreased fraction of wikis.
Arrhythmias and deterioration of the heart's performance appear in 70% of ailments and show great versatility. Frequent disturbances to the rhythm є supraventricular tachycardia, polytopy and groups of extrasystoles. The variability of arrhythmias is due to the severity of the heart and the sutta is overwhelmed by the forecast, especially in the sick with skeletal muscular mutilation in one hour, and may be the cause of the death of the raptus. The deterioration of the conductivity of the heart is manifested in the main increase in the interval P-Q, defects in the middle of the slurry conductivity and blockade of the anterior left side of the gus bundle. Signs of myocarditis are more likely to occur in ailments with symptomatology of polymyosis; myocarditis is associated with low vizivannya ailments. The dissection of the pericardium in the presence of adhesive and, earlier, exudative pericarditis with special pre-existing pericarditis occurs in 70-80% of the ailments, and often it is asymptomatic. In some cases, there is a significant vipit in the pericardium, which can lead to the tamponade of the heart. Sertseva's lack of development grows rarely, or in vipadkakh they appear to be refractory to therapy and an uncomfortable prognosis.
Changes of the heart may develop suddenly, in the case of pathology legends (Legenevian hypertension) or nirok (scleroderma nirkovy crisis).
Exercise nirok:
In preschool children, in the middle, 50% of the ailments have the same signs of nirk dysfunction: proteinuria, hematuria, a slight increase in creatinine levels in the blood, arterial hypertension. Slid vrahovuvati, as well as changes can be vicious and other reasons, such as heart failure, hypertension, nephrotoxicity in the first place.
- Virazhennya urazheniya nirok - scleroderma nirkovy crisis, develops in 5-10% of ailments, especially in ailments with a diffuse form of SSD. Characteristic manifestations of a scleroderma nirkovy crisis: є: a severely developed and progressive nirkovo deficiency; malignant arterial hypertension, associated with a high level of renin; normal slash siege or minor changes (microscopic hematuria and proteinuria). Proteinuria can appear back to the development of a nirk crisis, and it can appear during the development of this acceleration;
- Mentions, related to the lesions of nircoval vascular disease and arterial hypertension, including microangiopathic (non-immune) hemolytic anemia, thrombocytopenia, hypertensive retinal encephalopathy.
A special feature of the scleroderma nirkovy crisis is the raptor ear, without any precursors. Approximately 10% of ailments do not suffer from AT disease - so the titles of normotensive sclerodermic nirkovy crisis. Without lіkuvannya (wake up with a stretch of 1-2 mіs), a terminal nirkovo lack of development develops. Risk factors for scleroderma nirkovy crisis є diffuse form, intake of high doses of HA (more than 15 mg / day), AT to RNA-polymerase III.
Breakdown of the nervous system: Polyneuritic syndrome, which can be associated with dressings with Raynaud's phenomenon or primary lesions of peripheral nerves. Trigeminal sensory neuropathy is diagnosed in 10% of ailments and manifests itself in one or two-sided symptoms of an individual, sometimes in some cases with paresthesias. Patients with diffuse SJS often develop carpal tunnel syndrome. Before the first manifestations of SS, Sjogren's syndrome (20%), lesions of the thyroid gland (thyroiditis Hashimoto, de Quervain's thyroiditis) are diagnosed, before the development of hypothyroidism; Primary bilious cirrhosis in ailments with a form of SSD.

Laboratory Doslidzhennya:

- Zagalny blood analysis: hypochromic anemia, in addition to the adjustment of SHOE (approximately in half of the ailments), a decrease in hematocrit; ADVISION SHOE is not related to the clinical activity of the SSD and may be linked to the latent infection (zvvychay broncholegenic).
- Zagalny analysis of the sech: Hypostenuria, microhematuria, proteinuria, cylindruria, leukocyturia. The stage of the incidence of the sectional syndrome is variably fallen in the clinical form of the reduction.
- Biochemical analysis of blood: Characteristic changes in the daytime.
- IMUNOLOGICHNI DOSLIDZHENNYA... ANF ​​appears in 95% of patients with SJS; More important is the value of the so-calleds.
- ATScl-70, or AT to topoisomer-ze-1, more often appear with diffusion, less with limitanoy form of SSD. The presence of AT in the presence of - - - HLA-DR3 / DRw52 is 17 times greater than the development of lung fibrosis in SS. The AT caption is the main factor for the widening of the disease and the activity of the ailment. The appearance of ATScl-70 in ailments with the isolated phenomenon of Raynaud is associated with a further development of the SSD class.
- Anticentromeric AT (ACA) is found in 20% of sick SS, the head rank in case of limiting form. Also, 12% of ailments have primary biliary cirrhosis (half of the symptoms of SJS), even more rarely with chronic active hepatitis and primary lung hypertension. - ACA is seen as a marker for the development of the SSD when the Raynaud phenomenon is isolated.
- AT to RNA-polymerase III occurs in 20-25% of ailments, especially in a diffuse form and in diseases of nirok, associated with an unpleasant prognosis.
In addition to the list of anti-nuclear antibodies, with SSS with a lower frequency, anti-nuclear AT appears, including:
- AT to Pm-Scl occurs in approximately 3-5% of patients with SJS in the second place with polymyositis (SJS-on-lymiozit perekhresny syndrome);
- AT to Through RNP appear in 7% of ailments and associate with a diffuse form of ailment, primordial Legendary hypertension, lesions of skeletal mucosa and early debut of ailments;
- AT to U1 -RNP appear in the middle in 6% of SSS patients, associate with SSS-VKV with perehralny syndrome, arthritis, isolated hypertension and early onset of ailment.
RF appears in 45% of ailments, the head rank in cases of Sjogren's syndrome.

instrumental doslіgennya
Capillaroscopy of the nigth bed is characteristic of the SSD of the worm (dilatation and reduction of capillaries) at the early stage of ailment, a high sensitivity and specificity.
Oskіlki for SSc characteristic urazhennya bagatoh vіstseralnih systems SSMSC mozhut protіkati bezsimptomno (Especially on rannіy stadії hvorobi) for їh svoєchasnogo viyavlennya i otsіnki stage urazhennya neobhіdno provoditi vіdpovіdnі іnstrumentalnі doslіdzhennya, character i frequency yakih viznachayutsya klіnіchnoyu form, perebіgom hvorobi that neobhіdnіstyu control efektivnostі terapії (Table 1).
Table 1. Special dosage of internal organs in case of systemic scleroderma.

1. Biochemistry analysis of blood (creatine, K +, Na +, ALT, AST, spinal and direct bilirubin, lipid spectrum, glucose)

1. coagulogram
2. dobova proteinuriya
3. EXO-KG
4. UZDG Sudin Upper and Lower Kintsivok, Sudin Nirok
5. UZD OChP, nirok
6. FGDS, pH meter, pressure gauge to stravohodu
7. Roentgenoscopy of the stravohode, shlunk, two-fingered intestine with contrasting barium suspensions
8. CT legends
9. spirography
10. Biopsy of the shkirno-myazovy valve, nirki

Differential diagnosis

Differential diagnosis:
Differential diagnosis of SJS is carried out with some of the sick scleroderma groups, with a large number of external Raynaud's phenomenon and deterioration of internal organs.
· Diffuse eosinophilic fasciitis - induction of the shkiri to be repaired beforehand and / or even with extensions to the proximal to the proximal to the prominence and to the tulub; fingers of hands and faces become intact. It is characterized by the lesions of the shkiri for the type of "orange peel", with the original contractures, eosinophilia, hypergammaglobulinemia and the development of SHOE. Approximately in 1/3 of vipadk_v stitching is stitched with the preceding overwhelming physical damage or injury. The development of aplastic anemia is possible.
· Bushko's sclerodroma is a swirling foolishness in the area of ​​exposure, shoulder, shoulder girdle. It is not often the case that there is such a link with the previous infection of the upper nku-them.
· Surrounded by scleroderma - focal (plaque) and line ("blow to the pattern", hemiform) damage to the skin and soft tissue.
· Multifocal fibrosis. Main localization: retroperitoneal, intraperitoneal and mediastinal fibrosis; before - in the field of fibrosis in the legends, ochnitsi (pseudotumor of the eyes), thyroid glands (tireoiditRіdel) and іn. Dupuytren and keloid contracts are also seen up to the smallest forms. Frequently after 2-3 and more localization process.
· Tumor-associated (paraneoplastic) scleroderma - a variant of the paraneoplastic syndrome, which manifests itself as an overwhelming development of fibrosis in the periarticular tissues, contractures, or for the type of torpid before SSD therapy with overgrowth of the periphery.
· Pseudoscleroderma - shkiri changes, which are susceptible to congenital abnormalities of meta-bolism: porphyria, phenylketonuria, amyloidosis, Werner's syndrome, Rotmund's syndrome; diabetic pseudoscle-roderma; scleroxedeme and in.
Werner's syndrome (progeria of the grown-ups, a defect in the lamina gene) manifests itself as scleroderma-like wrinkles of the skin (especially kintzivok) and skeletal muscles, development of cataracts, hypogenitalism, early arteriosclerosis, insular deficiency; Sponsor more often with people in 20-30 years. Rothmund-Thomson syndrome (atrophic poikiloderma). Klіnіchno: poykіlodermіya oblichchya i kіntsіvok, FEB-sided cataract, distrofіya hair (nіgtіv i zubіv) gіpogonadizm, Porush-nya endohondralnogo okostenіnnya, ar-terіoskleroz i karlikovіst, gіperpіg-mentatsіya shkіri, teleangіektazії, atrofіchny dermatosis anemіya, pіdvischeny rizik osteosarcoma ... Synonyms: cataract, Rotmund dystrophy.
... Raynaud's phenomenon is one of the main symptoms, but there is a need for differential diagnostics of SJS with other systems that are affected by the resulting tissue: damage to secondary tissue, anti-synthetic dermatitis within the framework of polyitis /.

Likuvannya beyond the cordon

Take a lykuvannya in Korea, Izrail, Nimechchin, USA

Otrimati the consultation on medturism

lykuvannya

Cіlі lіkuvannya:
- prophylaxis and treatment of the sickness
- suppression of progression of fibrosis
- prevention and treatment of internal organs.
Likuvannya tactics:
... Early diagnosis and adequate therapy in the meaning of the world start the effectiveness of treatment and prognosis, especially in case of highly progressive diffusional SJS. Likuvannya is guilty of being as individualized as possible due to the clinical manifestations and activity of ailing.

Non-drug treatment:
Unique psychomotional navantazhen, trivial in pouring cold and vibrations, changing over to sleep. To reduce the frequency and intensity of attacks of vasospasm, we recommend wearing a warm dress, including the warmth of the lower blouse, headwear, outer shirts and mittens, replace the mitten. At the same time, recommend to the ailing one who is sick, from living with Kavi and drinking coffee, from a unique sympathomimetic (ephedrine, amphetamine, ergotamine), in-adrenergic blockers.

Drug treatment:
main directions drug therapyє Sudinna, antiepileptic and antiphysiological therapy, as well as treatment of visceral manifestations of SJS.
1. Sudin's therapy is based on the phenomenon of Raynaud. In addition, with SJS, the following LZ should be stashed:
Sildenafil is an inhibitor of phosphodiesterase, in a dose of 50 mg per day for the initiation of digital viral infections in ailments on SJS, in those who did not suffer from the effect of stagnation of calcium channel blockers.
Bosentan is a nonselective antagonist of endothelin-1 receptors; at a dose of 125 mg / dobu, 2 times lower the number of new digital virazoks.
2. Protistopal and cytotoxic drugs stagnate at the early (fiery) stage of SSD and early progression of illness:
· NPZP in standard therapeutic doses are indicated for the treatment of myazo-lumpy manifestations of SS, stiff subfebrile fever (temporal fever is uncharacteristic for SS).
· Glucocorticoids are indicated for progressive diffuse uterine disorders and obvious clinical signs of ignition activity (myositis, alveolitis, serositis, refractory arthritis, tendosynovitis) in small (not more than 15-20 mg / dose) doses. Acceptance of large doses of increased risk of development of normotensive scleroderma nirkovy crisis.
· Cyclophosphamide in the combination with the GK will be used for the treatment of IZL (div. Dali Porazka legeniv).
· Methotrexate is able to change the broadening and swelling of the shkiri, but does not infuse into the visceral pathology. Indicated for methotrexate є after SSA with RA or polymyositis.
Cyclosporine positively infuses on the dynamics of small children, however, nephrotoxicity and temporality of the development of a severe nirkovy crisis on aphids is seriously intertwined with the drug intake in SSD

1. Antimicrobial therapy is indicated at the early stage (stretching out the first 5 rocks of ailments) or in case of increased swelling and widening of the disease in ailments of diffusional systemic scleroderma. D-penicylamine is the main drug that induces fibrosis development. An effective dose of the drug is 250-500 mg / day.

1. Prednizolone, 5 mg, tab
2. Methylprednizolone 4 mg, 16 mg, tab.
3. Methylprednizolone 250mg, 500mg, vial.
4. Prednizolone, 30 mg, amp

1. D-pen_cylamin (cuprenil) 250mg, tab.

1. Cyclosporin 25 mg, 100 mg, caps
2. Cyclophosphamide 50 mg, dragee
3. Cyclophosphamide 200 mg, vial
4. Methotrexate 2.5 mg, table

1. Pentoxifilin 2%, 5 ml, amp
2. Vasaprostan 20 mg / 5 ml

1. Heparin 5000 MO, bottle
2. Clexane 0.4 ml syringe
3. Fraxiparine 0.3 ml, 0.4 ml, syringe
4. warfarin

hospitalization

Shown before hospitalization:
- For the first time, STS is revealed, especially the early stage of diffusion form.
- Multiple recurrent lesions of the skin and gangrene of the fingers of the hands and feet.
- Progressive lesions (fibrosing alveolitis, legeny hypertension), heart (exudative pericarditis), slunk-intestinal tract (abdominal pain, pseudo-ilus, malabsorption syndrome).
- Development of scleroderma nirkovy crisis (malignant hypertension, increased blood creatine).

prophylaxis

Prophylactic come in: Ethiology of the SSD is not home, in connection with which the primary prevention of ill health is not carried out. Prophylactic come in to be built before the fire starts getting sick and the development of the side effects of the medical therapy.

information

Dzherela and Literature

1. Protocols of the expert committee on nutrition development of health protection of the Ministry of Health of the Republic of Kazakhstan, 2013
   1. List of vicarious literature: 1. Rheumatic illnesses. Pid ed. J.H. Klippel, J.H. Stone, L.J. Cofford, P.H. White, 2012r. 2. Rheumatology, Ed. ON. Shostak, 2012r. 3. Diagnostics and treatment in rheumatology. Problematic pidhid, Pyle K., Kennedy L. Translation from English. / Ed. ON. Shostak, 2011r. 4. Rheumatology: Clinical recommendations / ed. Acad. RAMS E.L. Nasonov. - 2nd ed., Rev. і add. - M .: GEOTAR-Media, 2010 .-- 752 p. 5. Smolen J.S., Landewe R., Breedveld F.C. et al. EULAR recommendations for the management of rheumatoid arthritis withsynthetic and biological disease-modifying antirheumatic drugs. AnnRheumDis, 2010 rock; 69: 964-75. 6. Diffusion of ailments from the resulting fabric: a key for medicines / ed. prof. V.I. Mazurov. - SPb: SpetsLit, 2009r. 192 s. 7. West S.J. - Secrets of rheumatology, 2008r. 8. Rheumatology: national curriculum / ed. E.L. Nasonova, V.A. Nasonova. - Moscow: GEOTAR-Media, 2008 rock - 720 9.Federal capital of licenses of licars (formulary system). Issue VIII. Moscow, 2007. 10. Bulousov Yu.B. - Rational pharmacotherapy of rheumatic illnesses, 2005r. 11. Differential diagnosis of internal ailments: algorithmic input. P.M. Khili, E.J. Jacobson. Binom, Moscow, 2003.12. Vasculitis. Grinshtein Yu.I., Krasnoyarsk: IPK "Platina", 2003., 224 p. 13. Systemic lupus erythematosus - Donetsk: KP Region, 2003 - 464 p. 14. Rational pharmacotherapy of rheumatic illnesses. Essential for practical users. Edited by V.A. Nasonova, E.L. Nasonova. Litterra, Moscow, 2003. 15. Rheumatic ailments: nomenclature, classification, standards for diagnostics and treatment - V.N. Kovalenko, N.M. Fur coat - K.: TOV "Katran group", 2002. - 214 p. 16. Vasculitis and vasculopathy. E.L. Nasonov, A.A. Baranov, N.P. Shilkin. Verkhnya Volga, Yaroslavl, 1999. 17. Ridkis and atypical syndromes and incapacitation in the clinic of internal ailments - Gandzha I. M., Decik Yu.I., Peleschuk A.P. and in.; Pid ed. І. M. Gandzhi.- Kiev: I am healthy. 1983.- 544 p.

information

Assessment criteria for monitoring and auditing the effectiveness of the protocol

reviewer: Kushekbaeva A.E., Candidate of Medical Sciences, Associate Professor of the Department of Rheumatology, AGIUV

Results of the most recent re-census: the assessment is positive, it is recommended to be recommended before the weekend

List of Proceedings in the Protocol from the Values ​​of Qualitative Tributes
1. Togizbaev G.A. - Doctor of Medical Sciences, head post-state rheumatologist of the Ministry of Health of the Republic of Kazakhstan, head of the Department of Rheumatology of the AGIUV
2. Seisenbaev A.Sh. - Doctor of Medical Sciences, Professor
3. Aubakirova B.A. head postnatal rheumatologist in Astana
4. Sarsenbayuli M.S. - head postnatal rheumatologist of the Skhidno-Kazakhstan region
5. Omarbekova Zh.E. - Head post-state rheumatologist, Semey
6. Nurgalina S.M. - head postnatal rheumatologist of Zakhidno - Kazakhstan region
7. Kuanishbaeva Z.T. - head post-state rheumatologist of Pavlodar region

Letting the minds look at the protocol: the manifestation of new methods of diagnostics and treatment, destruction of the results of treatment, related to the provisions of the protocol.

Attached files

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• Vibir of lykarskiy zasobіv and їkh dozuvannya, is guilty of negotiations with Fakhіvts. Only a drink can indicate the need for a drink and a second dose for the sickness and become a sick person's body.
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Determination of shkiri - enhancements of the key signs of systemic vasculitis with lesions of small and middle caliber vessels. The nature of dermatological manifestations in the meaning of the world lies in the form of changes in sudin, which were present before the pathological process, and the immunological specificity of vasculitis. Histological advances in schooling are important for the diagnosis of visculitis, additional assistance in early differentiated diagnostics and timely recognition of adequate therapy. It is important for the doctor's staff to be suspicious, since dermatological manifestations show an important systemic vasculitis with multiple organ changes. This statistic presents clinical and histological data about the health problems of children with inferior systemic vasculitis, as well as the differentiated diagnostics algorithms.

Systemic vasculature is a heterogeneous group of ailments, the main morphological familiarity of such vasculature, and the spectrum of clinical manifestations is based on the type, size and localization of cases of abnormalities of the vertebral column. The prevalence of vasculitis in affected children rises from 15.4 to 29.7 per million of the population in Rik. More often women are sick, less people, grown-ups, a vignette is hemorrhagic vasculitis, which is more likely to develop (90%) in children. Shkirni may manifest as the first key symptoms of vasculitis, although the rule is that it stinks on the smallest systemic signs. Clinically vasculature in patients with children can be represented by a whole arsenal of non-specific or low-specific dermatological symptoms, which include pediatric institutes, purpura, palpation of papuli, lymphatic diseases, The detection of patients with systemic vasculitis does not contribute to the prognosis of illness, it can cause recurrent interruptions and it is important to get sick. I will look at a wide range of manifestations of the disease in case of systemic vasculitis, and the number of diseases, which can cause vasculitis, is not surprising, but in clinical practice it is often difficult to diagnose the diagnosis correctly. The most accepted pathogistological classification of systemic vasculature of the International Weather and Juvenile Conference in Chapel Hill, 2012 (Table 1).

The most frequent classification of vasculitis is the classification of the American College of Rheumatology (ACR), which is based mainly on key facts. However, offended by the classification of bully broken down for a group of patients with vasculitis, and not as a diagnostic criterion for a sick person.

Tilki deyaki vasculitis may cause pathognomonic clinics, instrumental (PET-angiography) and laboratory tests, once again the need for biopsy of the school for finding a precise method On the other hand, the histological confirmation of vasculature cannot be guarded from the history of the disease, clinical and laboratory conditions and / or even angiographic features.

Malunok 1.Histological classification (vibration of the optimal method of biopsy) of vasculitis in cases of shkiri (according to Carlson J.A., 2010)

With Schönlein-Genokh's ailment and a child's leukocytoclastic vasculitis, the surface of the shkiri is susceptible, while the vascular polyarteritis and the giant cell artery are the opposite of the fatty type of fatty cells, which are known to occur. A large number of other forms of vasculature, such as critical and ANCA-associative vasculature, can be hostile to others, as well as great vessels. Diagnostic value of biopsies of the school in the meaning of the world is to be found in the biopsies of the biopsies. For accurate diagnostics of all vasculitis, behind the blame leukocytoclastic and Schönlein-Henoch's ailments, it is necessary to carry out an incision (tissue development) or an excisional (adipose tissue) tissue clump.

A characteristic familiarity of the shkiri in ailments with vasculitis of the sudinum of the ear caliber є purple, yak palpation. Denmark is an element of shkіrny visipannya є as a result of the extravasation of erythrocytes through the vertebral column into the dermis. Important localization of purpuri є symmetrical patterns of lower patterns and back (photo 1). In case of leukocytoclastic vasculitis at the top of the purpura, there may be aseptic pustular elements (photo 2), enriched with a great number of reddened leukocytes. Purpura may be asymptomatic, in some cases, it may be a bit overwhelming, and it may cause hyperpigmentation.

Photo 1. Purpura of old age on homilka with hemorrhagic vasculitis

Photo 2. Purpura with pustular elements on homilka with leukocytoclastic vasculitis

The data on the association of singing options for cerebral viscosity with different types of vasculitis induction in table 2.

In 2009, a Japanese dermatologist T. Kawakami developed a diagnostic algorithm for small vasculature, which is based on immunological (ANCA, cryoglobulin, IgA) and histological data (Fig. 2).

Malunok 2. Diagnostic algorithm of primary small vasculature (after T. Kawakami, 2010)

The shortcomings of this algorithm є those who do not fail to protect the clinical picture of illnesses and diseases of the immune system (24% of the illnesses from the GPA are positive on the MPO-ANCA, 26% of the ills from the MPA and less than 5% of the ills from the ETSAH-3-positive on again to bring the importance of a comprehensive approach to the diagnosis of systemic vasculature.

university polyarteries

university polyarteries(UP) - a chain of systemic necrotization of vasculitis, which is characterized by lesions of the middle and other arteries with the form of microaneurysms, which can lead to the development of tissue ischemia and infarction.

Shkirnі show for the given literature is promoted in 26-60% of ailments with Uzelkovy polyarteritis. The dissection of the shkiri, as a rule, is supervised by the other systemic manifestations of UP (fever, thinning, more in myases, more in slopes, peripheral neuropathy). Due to the data of Agard C. and spіvavt, the prevention of shkіri (purpura, pedshkіrnі vuzli) had the first symptoms in 11% of patients with Nodular polyarteritis. Systemic manifestation can appear only after 1-20 rocky times when the shkirny visipanny appears. Most frequent dermatological manifestations of polyarthritis in nodules, infarction, infarction, often livedo, pediatric universities and ischemic changes in the distal phalanges of the fingers (photo 3). Naybіlsh widened the localization of the shkіrny visipannya - the lower pictures (95%). Pedagogical universities from yaskravo-chervony to Cyanotic color may have a size of 0.5-2 cm, as a rule bilateral, localized on homilk and stegna, grown - hands, tulubs, heads, shia, sidnitsyah. At the junction with the ishemian universities, there are bends (photo 4). Most of the time, it can be done independently, or one hour at a time with the educational institutions. The localization of livedo - lower and upper kintsіvki, and later - tulub has been expanded. Livedo is a macular ring-shaped system that can be used to create a cyanotic bar. The pathognomonic symptom of UE is the appearance of the so-called "sparse" or tree-like livedo, which appears as a frequent form of visipa (tree-like livedo is stored with rose or irregular photos) (5) (5). Unimportant in the classroom, in the literature, the term "sitchate livedo" is often used for the meaningful life. In children with ailments, Uzelkovym polyarteritis is found to have an atrophic, acute form of cicatrix (bila atrophy of shkiri).

Photo 3. Gangrene of distal phalanges of fingers in an ailing patient with Nodular polyarteritis

Photo 4. Virazki homilok at the sick man with Uzelkovy polyarteritom

Photo 5. Tree-like livingdo at the sick man with Uzelkovy polyarteritom

The largest manifestations of polyarthritis in nodules can be kropivyanka, transient erythema, superficial phlebitis, Raynaud's syndrome and infantile hemorrhage. Empty worms are characteristic for UP and as a rule are found in the result of secondary detection of necrotic wines.

According to the data of one retrospective dosage, the defeat of the children was spared in half (52%) of the ailments with Nodular polyarteritis (n = 112). Typical manifestations of boules of pedigree vosliks and vigorous-necrotic snakes (in 20.7% of ailments), livedo (in 15.5% of ailments) and polymorphic visip (13.8%). The earlier developed elements of the battle of the school (Figure 3).

Malunok 3. The structure of shkіrnyh manifestations in ailments Nodular polyarterіtom in the debut of ailing

Classical histological familiarity with the polyarteritis of the university is the manifestation of necrotized firing of mid-diameter vessels (photo 6). Іnu chotiri gistolіgіcheskіe stage in the development of university polyarthritis: degenerative, the stage of state of fire, development of granulation tissue і thermal. The degenerative stage includes coagulation necrosis of the middle shell of the sudin, fibrinous exudate near the outer elastic membrane, neutrophilic inflammatory and partial deformations of the lateral membrane and internal Stadіya GOSTR broken-winded harakterizuєtsya neutrophilic, lymphocytic i eozinofіlnoї іnfіltratsієyu, povnoї deformatsієyu vnutrіshnoї elastichnoї membrane fіbrinoznimi eksudat vsієї sudinnoї stіnki of Povny deformatsієyu serednoї Ilya Obolonkov, prolіferatsієyu fіbroblastіv, nabryaklimi zmіnami navkolishnogo spoluchnoї tkanini i totalnoї oblіteratsієyu prosvіtu sudin formuvannyam of fibrin clots. At the stage of the development of granulation tissue, lymphocytes replace neutrophils, the emerging granulation tissue, which hunts the middle and late shell of the judge and can penetrate through defects in the inner lining of the elastic membranes in The thermal stage includes insight into the vertebral stage of scar tissue and perivascular proliferation of fibroblasts.

Photo 6. Vuzlikovy polyartery. Necrotic vasculitis of mid-caliber sudin (according to Carlson J.A., 2010)

With acute lesions of histological development, visceral dermatitis of the middle diameter of the pediatric adipose tissue with neutrophilic infiltration, leukocytoclase, necrotizing endothelial necrosis Pediatric universities are histologically represented by neutrophilic vasculitis of the oysteroid type with an important localization in the areas of bifurcation.

microscopic polyangias

microscopic polyangias(MPA) - systemic vasculitis with lesions of other vasculature (arterioles, capillaries and venules) without post-vascular granules. Microscopic polyangiitis є is characterized by the development of segmental necrotic glomerulonephritis, hemoptysis and association with ANCA (26% of ailments are positive for antibodies to PR-3 and 58% of ailments are for antibodies to MPO). In a large number of patients with microscopic polyangiitis, the development of pulmological and nephrological symptoms is overwhelmed by arthralgia, malignancy and constitutional symptoms (feverishness, decreased body mass).

Dermatological manifestations appear in 15% of ailments in the onset of MPA and up to 65% of ailments in a disease. Some typical dermatological signs of microscopic polyangiitis are purpura, which can be palpable and appear in about 50% of ailments, and localize on lower symptoms. Some dermatological manifestations include pediatric hemorrhage, pediatric vascular disease, palmar erythema, livedo, hemorrhagic bully, vesicles, infarction, cytoplasmic hemorrhage, telesectomy. Behind the figures, in the middle of the microscopic manifestations of the microscopic polyangiitis (n = 14), purpura, palpation, vigorous-necrotic snakes, livedo are more often observed.

Classical histological signs of the MPA for the data of the biopsy of the school є neutrophilic vasculitis of other sudin dermis and pediatric fatty cells. It is rare to develop a median diameter to the pathological process. Other histological signs include lymphocytic perivascular disease of the upper dermis, reduction of lymphocytic and neutrophilic perivascular disease of the middle The tree-like livedo is histologically represented by vasculitis of the swelling spheres of the dermis and the adipose tissue. Determination of the vessels of the pulmonary caliber by the diagnostic criteria for MPA, which includes the diagnosis of the polyarteritis of the nodules. Histological differentiation of familiarity between GPA and MPA є visibility of the formation of granules with MPA.

Granulomatosis with polyangiitis (Wegener's granulomatosis)

Granulomatosis with polyangiitis(GPA) - systemic vasculitis, which is known from the classification of the International Weather and Juvenile Conference in Chapel Hill, including the following three triad: granulomatous inflammation of the respiratory tract, necrotizing vasculitis of the middle and other malformations. However, only 16% of ailments from the GPA show all three classification criteria. Typical laboratory manifestations of HPA are positive for antibodies up to PR-3 (66%) and for antibodies up to MPO (24%). Klіnіchnoї protyagom GPA often suprovodzhuєtsya konstitutsіynimi Wink (lihomanka, vtrata masi tіla) artralgіya, mіalgіya i urazhennyam verhnіh dihalnih shlyahіv (rinіt, sinusitis, virazki slizovoї nosovoї porozhnini i company, perforation nosovoї partition sedlopodobnaya deformatsіya nasal granulomatous broken-winded traheї of formuvannyam sobglotochnogo stenosis ).

Determination of the shkіri in the ailments of the GPA begins to develop, according to the data of the young people, with a frequency of 14 to 77% and in 10% of the ailments the first symptoms of illness. A common element of shkіrny visipannya with GPA is purpura, yak palpation, with localization on the lower pictures.

Papulo-necrotic changes are seen in ailments with GPA more, or more specific symptom in cases of purpura, which can be palpable. Shkirnі postasudinal necrotic granulomas or papulo-necrotic vapors can be found in the typical localization of rheumatoid universities (photo 7). I will look at the positivity of one third of the ailments on HPA on the rheumatoid factor and the presence in the debut of suglobous syndrome, often such ailments arise to diagnose rheumatoid arthritis. In such cases, it is more important to carry out a differentiated diagnosis of antibodies to cyclic cytrullin bile, which do not appear in patients with HPA.

Photo 7. Papulo-necrotic visip on the lіktі at the GPA ailment

Prior to the first manifestations of the disease in patients with ailments, the GPA є pediatric universities, vesicles, digital infarctions, infarctions, creeps, how to foul pyoderma gangrenosum, and poly. On the basis of a high school polyarticity, GPA is not characterized by the presence of livedo. In the case of HPA ailments, who were passed over visually (n = 25), the disease was observed in 52% of cases, including necrotic papules - in 28%, digital infarction - in 16%, polymorphic visip - in 12%.

Іnu chotiri of histological changes of biopsies of shkiri among ailments of the GPA:

1. Necrotizing neutrophilic vasculitis of sudin dermis of the middle and middle caliber.
2. Palisadna granuloma with the central nucleus, is represented by basophilic collagen, excreted by histiocytes and neutrophils (the so-called "blakytna" granuloma).
3. Granulomatous vasculitis with perivascular lymphogistic cells and infiltrates and the manifestation of infiltrating blood vessels of the myazoid type of pediatric adipose cells and giant cells.
4. Perivascular infiltration by atypical lymphocytes.

The biopsy of shkiri in GPA ailments often demonstrates granulomatous changes and rarely signs of vasculitis.

Eosinophilic granulomatosis with polyangiitis (Charg-Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis(EGPA) - systemic vasculitis, which is characterized by the manifestation of bronchial asthma (as a rule with other onset), allergic symptoms (allergic rhinitis, nasal emptying polypy), peripheral mid-vascular eosinophilus In 40% of EGPA ailments, antibodies to MPO appear, in 5% of ailments - AT to PR-3. Picking up shkiri, yaku supervise peripheral neuropathy - a characteristic sign of Churg-Strauss syndrome. Some of the most important signs include infirmities, abdominal pain, intestinal problems, arthralgia, maladies and constitutional symptoms. Disorder of shkiri is indicated in 40-75% of ailments from the EGPA and in 6% of ailments with the first symptoms of illness. Yak for some ANCA-associative vasculitis, for EGPA, characteristic lesions of shkiri є purpura, like palpation, with a typical localization on the lower pictures, like to appear in half of the ailments about shkids. Pediatric universities and papules-necrotic changes of the lower images, rozginal lyktyovy surfaces, fingers and hairy part of the head appear in a third of the sick. Prior to the dermatological manifestations of the EGPA, they are often referred to as livedo, virazki, vesicles, multiforme erythema, digital arteritis, panniculitis, and incrustation. Among the ailments of the EGPA shkirny urazhennya appeared in 36% of cases, overwhelmingly necrotic changes, digital arteries, purpura, palpation, panniculitis. The relatively low frequency of dermatological manifestations may be associated with this, but a large number of ailments with cystic vasculitis were treated in the field of a rheumatologist, and they were also treated with glucocorticoids, designated as a pulmonary drug.

The biopsy of the school will show the three main histological features of the EGPA:

1. Eosinophilic and neutrophilic vasculitis of the common and middle caliber of superficial and middle spheres of the dermis.
2. Interstitial analysis of dermis eosinophils.
3. Formation of "chervonoi" granulomas (photo 8). "Chervona" granuloma is deposited from the central nucleus, represented by the products of eosinophilic growth and collagen fibers, and hystiocytes distributed along the periphery.

Photo 8. Eosinophilic granulomatosis with polyangiitis. Vasculitis of mid-diameter sudin with eosinophilic infiltrates (according to Carlson J.A., 2010)

The diagnosis of shkiri in ailments on systemic vasculitis is often referred to as clinical signs of a sickness. The range of small lesions should be wide, with a wide variety of dermatological changes that are specific for certain forms of systemic vasculature (for example, for nodular polyarthritis - tree-like livedo, gangrene of distal palatal diseases) For early diagnosis and recognition of adequate therapy for systemic vasculitis with dermatological manifestations, in addition to clinical symptoms and immunological findings, it is more important to carry out a histological examination of the cells of the child.