50. SYSTEMIC SCLERODERMA

Chronic polysyndromic connective tissue disease occurs in young and middle-aged women.

Classification. When refining the forecast, the classification of N. G. Guseva (1988) is used:

1) downstream;

2) according to the stage of the disease;

3) according to the degree of activity (see Table 1).

Table 1

Determination of process activity in systemic scleroderma

Clinic. The main syndromes are vascular (Raynaud's syndrome), trophic disorders (rat bite symptom, ulcerative lesions), cutaneous (mask-like appearance of the face, skin thickening), articular-muscular (arthralgia in large joints, polyarthritis, fibrosing myositis, muscle weakness), visceral (lesions heart - non-coronary sclerosis, myocarditis, mitral disease, pericarditis, lungs - pneumosclerosis, digestive tract - dysphagia, gastritis, enteritis; kidney - chronic nephritis, acute scleroderma nephropathy, nervous system - polyradiculoneuritis, meningoencephalitis), asthenovegetative (weight loss, weakness, increased fatigue , unstable fever).

In the chronic course, Raynaud's syndrome progresses. Over time, changes appear in the nail phalanges, thickening of the skin, periarticular tissues, the formation of contractures, osteolysis, and slowly progressive changes in internal organs.

In subacute course, there is a dense swelling of the skin, recurrent polyarthritis, myositis, damage to internal organs.

In an acute course, a rapidly progressive variant of the course is fibrinous and visceral lesions already in the first year of the disease, the formation of a scleroderma kidney.

Diagnostic research. The examination algorithm corresponds to diagnostic tactics in connective tissue diseases.

Criteria for diagnosis are skin changes in the form of dense edema, induration and atrophy, Raynaud's syndrome, osteolysis, soft tissue calcification, joint-muscular syndrome with the development of contractures, basal pneumonitis, macrofocal cardiosclerosis, damage to the digestive tract, esophagus, true scleroderma kidney, hyperpigmentation skin, trophic disorders, immune disorders, the appearance of antinuclear antibodies with an increase in their titer.

If four or more signs are present, the diagnosis is considered reliable.

Treatment. Hormones, immunosuppressants, 4-aminoquinoline drugs, non-steroidal anti-inflammatory drugs, anticoagulants, antiplatelet agents are used - as in other connective tissue diseases, anabolic hormones for cachexia. Physiotherapy is used.

Forecast. In chronic course, the prognosis is relatively favorable, with stabilization, in acute and subacute course - unfavorable.

Author: Myshkina A.K.

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