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Internal illnesses. Systemic scleroderma (lecture notes)

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LECTURE No. 42. Systemic scleroderma

This 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 7).

Table 7. Determination of prognosis in systemic scleroderma

Clinic. The main syndromes are vascular (Raynaud's syndrome), trophic disorders (a symptom of a rat bite, ulcerative lesions), skin (masculine face, skin thickening), musculoskeletal (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; kidneys - chronic nephritis, acute scleroderma nephropathy, nervous system - polyradiculoneuritis, meningoencephalitis), asthenovegetative (weight loss, weakness, increased fatigue , unstable fever).

RџSЂRё chronic course progression of Raynaud's syndrome. Over time, there are changes in the nail phalanges, thickening of the skin, periarticular tissues, the formation of contractures, osteolysis, slowly progressive changes in internal organs.

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

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

Additional diagnostic study. The examination algorithm corresponds to diagnostic tactics in connective tissue diseases.

Criteria for making a diagnosis are skin changes in the form of dense edema, induration and atrophy, Raynaud's syndrome, osteolysis, soft tissue calcification, musculoskeletal syndrome with the development of contractures, basal pneumonitis, macrofocal cardiosclerosis, lesions of the digestive tract, esophagus, true scleroderma kidney, skin hyperpigmentation, 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.

Differential diagnostics. Should be carried out with systemic connective tissue diseases, rheumatoid arthritis, infectious myocarditis, atherosclerotic cardiosclerosis, pneumoconiosis, sarcoidosis, tuberculosis, chronic pneumonia, esophageal cancer.

Treatment. For the treatment of the disease, hormones, immunosuppressants, 4-aminoquinoline drugs, non-steroidal anti-inflammatory drugs, anticoagulants, antiplatelet agents are used - as in other connective tissue diseases, anabolic hormones in cachexia. Physiotherapy is used: electrophoresis with lidase, ultrasound, paraffin applications; massage, physiotherapy exercises, radon, hydrogen sulfide baths in chronic course.

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

Author: Myshkina A.A.

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