SCLERODERMA - a progressive disease characterized by changes in connective tissue with the development of sclerosis and obliterating lesions of arterioles; manifested by skin tightening, and with the systemic nature of the process and damage to internal organs. Systemic scleroderma is a serious disease with a polymorphic clinical picture, including damage to the skin, joints, internal organs (heart, lungs, gastrointestinal tract, kidneys) against a background of vascular and trophic disorders. It belongs to the group of diffuse connective tissue diseases. In etiology, genetic and infectious factors may be relevant. The disease is provoked by cooling, injuries, foci of chronic infection, drug intolerance, endocrine changes (for example, after childbirth, in the menopause), etc. In the pathogenesis of the disease, great importance is attached to changes in the metabolism of connective tissue, immune disorders and damage to the microvasculature.
Mostly women are sick, most often at the age of 20-50 years. The disease usually develops gradually: vasomotor disorders of the type of Raynaud’s syndrome (see Raynaud’s disease), joint pain, dense swelling of the skin, and later internal organs are affected. Skin lesion, passing the stages of edema, compaction and atrophy, is noted in most patients and is localized mainly in the face (masking) and brushes - sclerodactyly. Hyperpigmentation of the skin and telangiectasia on the face and chest are often observed. Phenomena of Raynaud's syndrome may be accompanied by severe trophic changes, repeated ulcerations at the fingertips; sometimes gangrene of the fingers develops. Most patients have arthritis with damage to small joints - from light arthralgia to severe joint deformation with the development of persistent contractures. In some cases, there is osteolysis (limited bone resorption) of the nail, and sometimes the middle phalanges of the fingers, less often the legs, with the shortening and deformation of the corresponding fingers; Calcium salts are deposited in the subcutaneous tissue of the fingers and periarticular tissues.
Damage to the lungs occurs in most patients, can manifest as shortness of breath and is characterized by the gradual development of pneumosclerosis.
Myocardial damage is observed in the form of focal or diffuse cardiosclerosis, manifested by rhythm disturbances and heart failure, less often - endocardium with the formation in some cases of heart disease (usually in the form of mitral valve insufficiency), pericardial changes are detected. Damage to the organs of the gastrointestinal tract (most often the esophagus) develops in about half of cases and is manifested by a violation of swallowing, heartburn. In some cases, abdominal pain, persistent constipation , and sometimes diarrhea are noted. Kidney damage is relatively rare, manifested by arterial hypertension, proteinuria; in acute and subacute course of the disease, the development of acute renal failure is possible. Often the functions of the endocrine (thyroid, sex glands, adrenal glands) and nervous (often peripheral and autonomic) systems are disturbed. Hair loss and significant loss of body weight (up to 10 - 20 kg or more) are observed.
Treatment is carried out for a long time (for years), mainly on an outpatient basis. Of the drugs used are D-penicillamine, corticosteroids, aminoquinoline derivatives (delagil, etc.), vasodilators, non-steroidal anti-inflammatory drugs, drugs that improve the state of microcirculation; locally use dimethyl sulfoxide, hyaluronidase. In the chronic course, balneotherapy (hydrogen sulfide, radon baths) is indicated, in case of joint damage - physiotherapy exercises and massage.
Depending on the speed of progression and activity of the process, acute (rarely observed), subacute and chronic course of the disease is distinguished. The prognosis for acute and subacute course is often unfavorable, for chronic it improves.
Prevention of exacerbations consists in the exclusion of provoking factors, the employment of patients and systematic observation in order to conduct maintenance therapy, to prevent further generalization of the process.