Scleroderma systemic

Scleroderma systemic is a chronic systemic disease of connective tissue and small vessels with widespread fibrotic-sclerotic skin and stroma changes of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome.

The etiology is unknown. It is provoked by cooling, trauma, infection, vaccination, etc.

In pathogenesis of leading importance is the violation of collagen metabolism, associated with the functional hyperactivity of fibroblasts and smooth muscle cells of the vascular wall. No less important factor of pathogenesis is the disturbance of microcirculation caused by damage to the vascular wall and changes in intravascular aggregate properties of blood. In a sense, systemic scleroderma is a typical collagen disease associated with excessive collagen formation (and fibrosis) by functionally inferior fibroblasts and other collagen-forming cells. Family-genetic predisposition is important. Women are sick 3 times more often than men.

Symptoms, course. Usually the disease begins with Raynaud's syndrome (vasomotor disorders), trophic disorders or persistent arthralgias, weight loss, fever, asthenia. Starting with any one symptom, systemic scleroderma gradually or fairly quickly acquires the features of a multi-syndrome disease.

The defeat of the skin is a pathognomonic sign of the disease. This is a common dense swelling, in the future - densification and atrophy of the skin. The greatest changes undergo skin of the face and limbs; Often the skin of the entire body is dense. At the same time, focal or widespread pigmentation with sites of depigmentation, telangiectasia develops. Characterized by ulcers and pustules at the fingertips, long-term healing and extremely painful, deformation of the nail, hair loss until baldness and other trophic disturbances.

Fibrotic interstitial myositis often develops. Muscle syndrome is accompanied by myalgia, progressive condensation, then muscle atrophy, muscle strength decrease. Only in rare cases there is an acute polymyositis with pain, swelling of the muscle, etc. Fibrotic changes in muscles are accompanied by tendon fibrosis, which leads to muscle-tendon contractures - one of the reasons for the relatively early disability of the patients. The defeat of the joints is mainly due to pathological processes in the periarticular Tissues (skin, tendons, articular bags, muscles). Arthralgia is observed in 80 - 90% of patients, often accompanied by severe deformity of the joints due to proliferative changes of the periarticular tissues; X-ray examination does not reveal significant destruction. An important diagnostic sign - osteolysis of the terminal, and in severe cases and medium phalanges of the fingers, less often of the legs. The deposition of calcium salts in the subcutaneous tissue is localized mainly in the region of the fingers and periarticular tissues, expressed as painful uneven formations, sometimes spontaneously opening with the rejection of crumblike calcareous masses.

The defeat of the cardiovascular system is observed in almost all patients: the myocardium and endocardium are affected, rarely the pericardium. Scleroderma cardiosclerosis is clinically characterized by pain in the heart, dyspnea, extrasystole, muted tones and systolic murmur at the tip, extending the heart to the left. Radiologic examination shows weakening of pulsation and smoothness of the heart contours, radiographic imaging - mute zones in the areas of large-sclerotic cardiosclerosis; And in the most severe cases an aneurysm of the heart is formed in connection with the replacement of fibrous muscle tissue. On the ECG, there is usually a decrease in voltage, conduction disturbances, up to the atrioventricular block; Infarct-like ECG occurs with the development of massive foci of fibrosis in the myocardium. If the process is localized in the endocardium, it is possible to develop sclerodermal heart disease and defeat the parietal sinocard. The mitral valve usually suffers. Sclerodermal heart disease is characterized by benign flow. Cardiac insufficiency develops rarely, mainly with the common defeat of the heart muscle or all three of its membranes.

The defeat of small arteries, arterioles causes such peripheral symptoms of scleroderma as Reynaud's syndrome, gangrene of fingers. The defeat of the vessels of internal organs leads to severe visceral pathology - hemorrhages, ischemic and even necrotic changes with a clinical picture of severe viscera (disintegration of lung tissue, "true scleroderma kidney", etc.). Vascular pathology determines the rapidity of the course of the process, its severity and often the outcome of the disease. At the same time, it is possible to damage large vessels with a clinical picture of obliterative thromboangiitis; Develop ischemic phenomena, and often gangrene of the fingers of the toe, migrating thrombophlebitis with trophic ulcers in the region of the feet and shins, etc.

The defeat of the lungs in the form of diffuse or focal pneumofibrosis, mainly the basal parts of the lungs, is usually accompanied by emphysema and bronchiectasis, and often adhesive pleurisy. Shortness of breath, difficulty in deep inspiration, hard breathing, wheezing with auscultation of the lungs, box tone of percussion sound, decrease in vital capacity up to 40-60% due, bilateral reinforcement and deformation of the lung pattern sometimes with a fine-meshed structure (honeycombs); When X-ray examination - signs that usually characterize scleroderma pneumofibrosis. Kidney damage manifests more often than focal nephritis, but diffuse glomerulonephritis with hypertensive syndrome and kidney failure is possible. With a rapidly progressing course of systemic scleroderma, a "true scleroderma kidney" often develops, due to damage to the kidney vessels, leading to focal necrosis of the cortex and renal failure. Esophageal damage, manifested by dysphagia, enlargement, weakening of peristalsis and rigidity of the walls with slowing down of passage of barium during X-ray examination, is observed very often and has an important diagnostic value. Often in the lower part of the rigid esophagus peptic ulcers are formed. In connection with the vascular lesion, ulceration, hemorrhage, ischemic necrosis and bleeding in the digestive tract may develop.

The defeat of the nervous system is manifested by polyneuritis, vegetative instability (violation of sweating, thermoregulation, vasomotor reactions of the skin), emotional lability, irritability, crying and hypothetical insomnia. Only in rare cases there is a picture of encephalitis or psychosis. Symptomatic of cerebral vascular sclerosis is possible due to their sclero- and dermis involvement, even in young people. There is a lesion of reticuloendothelial (polyadenia, and in a number of patients and hepatosplenomegaly) and endocrine (plurigandular insufficiency or pathology of one or other endocrine gland) systems.

More often there is a chronic course, the disease lasts for dozens of years with minimal activity of the process and the gradual spread of lesions to various internal organs, the function of which is not disturbed for a long time. Such patients suffer mainly from skin lesions, joints and trophic disorders. Within the framework of chronic systemic scleroderma, CRST syndrome (calcinosis, Reynaud's syndrome, sclerodactyly and telangiectasia) is distinguished, characterized by a long-term benign course with extremely slow development of visceral pathology. In subacute flow, the disease begins with arthralgia, weight loss, visceral pathology rapidly builds up, and the disease acquires a steadily progressing course with the spread of the pathological process to many organs and systems. Death usually occurs 1 to 2 years after the onset of the disease.

Laboratory data are not typical. Usually moderate normo- or hypochromic anemia, moderate leukocytosis and eosinophilia, transient thrombocytopenia are observed. ESR is normal or moderately elevated in chronic course and significantly increased (to 50-60 mm / hr) with subacute. Treatment: use of anti-inflammatory and restorative drugs, restoration of lost functions of the musculoskeletal system.

Active anti-inflammatory therapy with corticosteroids is indicated mainly in subacute flow or during periods of pronounced activity of the process in chronic course. Prednisolone 20-30 mg give for 1 to 1.5 months until a pronounced therapeutic effect is achieved, then it is very slowly reduced, the maintenance dose (5-10 mg of prednisolone) is used for a long time, until a persistent effect is obtained. In the period of lowering the doses of hormonal drugs, non-steroidal anti-inflammatory drugs can be recommended. D-penicillamine is prescribed on 150 mg 3 - 4 times a day with a gradual increase to 6 times a day (900 mg) for a long time, at least a year; Especially shown with rapid progression of the disease; The most serious complication is nephrotic syndrome, which requires immediate discontinuation of the drug; Dyspeptic disorders decrease with a temporary withdrawal of the drug, a change in taste can be corrected by the appointment of vitamin B. Aminohinoline preparations are shown in all variants of the course. Delagil (0.25 g once a day) or plakvenil (0.2 g 2 times a day) can be prescribed for a long time, for years, especially with the leading articular syndrome.

In recent years, calcium channel blockers, corinfar (nifedipine), have been widely used for 30 to 80 mg / day for months with good tolerability. With a "true scleroderma kidney" - plasmapheresis, a prolonged intake of captopril 400 mg per day (up to 1 year or more). In chronic course, lidase (hyaluronidase) is recommended, under the influence of which stiffness decreases and mobility in the joints increases, mainly due to softening of the skin and underlying tissues. Lidaz is injected every other day with 64 UE in a 0.5% solution of novocaine sc (12 injections per course). After 1 - 2 months, a course of treatment with lidase can be repeated (4-6 courses a year). With a pronounced angiospastic component (Raynaud's syndrome), repeated angiotrophin regimens (1 ml SC, for 30 injections), kallikrein-depot, andecalin (1 ml IM, for a course of 30 injections) are shown.

In all cases, active vitamin therapy, ATP, is recommended. In chronic course balneotherapy (conifers, radon and hydrogen sulphide baths), paraffin and mud applications, electrophoresis of hyaluronidase, applications with 30-50% solution of dimethylsulfoxide (20 - 30 sessions) on the affected limbs are shown. Of great importance are exercise and massage. In subacute flow, morning hygienic gymnastics and an active position in bed are recommended, with chronic - persistent and prolonged use of medical gymnastics in combination with massage and various labor processes (molding from warm paraffin, weaving, sawing, etc.).