Systemic Scleroderma

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Scleroderma is a disease that affects the connective tissue of the body, in which there is a thickening (hardening) of the skin and blood vessels. As a rule, the pathological process affects the entire body as a whole, and then they talk about systemic scleroderma. Rare cases when changes concern a small area of tissue are called focal scleroderma.

The disease affects people of all age groups, including children. Women suffer from systemic scleroderma three times more often than men. This is a fairly rare disease, occurring in 3 in every 100,000 people.

The causes of systemic scleroderma

The origin and causes of systemic scleroderma are unknown. Currently, many doctors are inclined to consider the disease as a manifestation of a viral pathology that triggers an autoimmune reaction, since a large number of virus-like particles are found in the tissues of people with systemic scleroderma, and an increased amount of antibodies to them is found in the blood. Hereditary predisposition matters, people from the same family are more often sick. Of the contributing factors, the following are noted:

Disorders of the nervous and endocrine systems;
Injury;
Postponed stress;
Vaccination;
Hypothermia;
Infection;
Taking certain medications (cytostatics);
Prolonged contact with certain chemicals (silica dust, vinyl chloride, coal dust, some organic solvents).

Systemic scleroderma symptoms

Systemic scleroderma refers to polysyndromic diseases, since as a result of damage to the blood vessels, all organs and tissues of the body are affected without exception. The most characteristic manifestations of the skin: first, a dense edema of the skin forms, which becomes more and more dense (sclerosed), then at this place the skin atrophies. Such phenomena occur throughout the body, but are most noticeable on the face and limbs. Hair falls out on the affected areas, the skin becomes discolored, the nail plates are deformed on the extremities, painful abscesses appear on the tips of the fingers, turning into ulcers, and then scars. The face takes on a motionless, mask-like appearance, it is difficult to open the mouth. The skin is dry, dense, "mummified".

Lesions of the musculoskeletal system are expressed in myositis (muscle inflammation) caused by the replacement of normal muscle and tendon tissue with fibrous tissue, which leads to contractures. The joints are also limited in movement, morning stiffness syndrome appears, the joint tissue is deformed, pain occurs, and calcium salts are deposited. One of the characteristic signs of systemic scleroderma is osteolysis of the terminal phalanges of the fingers, as a result of which the fingers become shorter.

Raynaud's syndrome is characteristic of the symptoms of systemic scleroderma: a spastic attack, as a result of which the fingers or toes become cold, numb, a tingling sensation appears in them, while they become white or even bluish. This happens under the influence of stress or temperature exposure, and can last from several minutes to several hours. At the end of the attack, the cyanotic skin turns red, there is a feeling of heat and pain.

The defeat of internal organs in systemic scleroderma manifests itself in the form of a total defeat of all systems due to circulatory disorders. Heart diseases develop (cardiosclerosis, myocarditis, endocarditis), kidneys (chronic nephropathy), gastrointestinal tract (esophageal stenosis is characteristic), etc. are affected. The nervous system and the mental state of a person also suffer.

The nature of the course and prognosis of systemic scleroderma

Systemic scleroderma can occur as an acute, subacute, and chronic disease.

The acute course is the most malignant, characterized by the rapid development of diseases of the internal organs and a constant increase in symptoms. In this case of systemic scleroderma, the prognosis is poor, since a year after the onset of the development of the disease, a critical violation of the functions of internal organs occurs.
Subacute course. A rapidly developing lesion of the skin, muscles and joints, with slow fibrosis of the internal organs. Symptoms increase steadily, but slowly. In this case of systemic scleroderma, the prognosis is moderately favorable and depends on the degree of generalization and activity of the process.
Chronic course. In the case of a chronic course of systemic scleroderma, the prognosis is the most favorable, since the symptoms grow very slowly, over years and decades, and the organs do not lose their functions for a long time.

In addition to the course, the clinical form of the disease and the general condition of the patient are important for the prognosis of systemic scleroderma.

Diagnosis of systemic scleroderma

Diagnosis of systemic scleroderma is carried out on the basis of a characteristic clinical picture and the presence of diagnostic criteria - manifestations characteristic of this particular disease. To diagnose systemic scleroderma, a large and several small diagnostic signs are required. According to the system developed by the American Rheumatological Association, the diagnostic criteria for systemic scleroderma are as follows:

Large (main): scleroderma skin lesion of the trunk with the characteristic signs described above (symptoms of systemic scleroderma).
Small (additional): characteristic lesions of the fingers (sclerodactyly), symmetrical basal pneumosclerosis (fibrous lesions of the lungs), scarring on the fingerpads, Raynaud's syndrome.

Also, for the diagnosis of systemic scleroderma, laboratory tests of blood, urine, immunodiagnostics, X-ray examination of bones and joints, ultrasound of internal organs, cardiology are performed, that is, a full range of examination of those organs from which there are the most striking manifestations.

Systemic scleroderma treatment

Treatment of systemic scleroderma is mainly symptomatic, aimed at alleviating the patient's condition and reducing the activity of the disease. In acute and subacute course, high doses of hormonal anti-inflammatory drugs are used to treat systemic scleroderma. In all other cases, general strengthening agents, vitamin therapy, immunocorrection are used. To weaken fibrosis, drugs that soften the connective tissue (lidases) are used. Also used drugs that improve the work of the microvasculature.

Physiotherapy and physiotherapy exercises are of great importance. Physiotherapy procedures are used as fortifying and stimulating metabolism in damaged tissues, which prevents their atrophy. For the same purpose, specially developed exercise therapy exercises are used. Treatment of systemic scleroderma should be continuous, general strengthening and developing the musculoskeletal system should become a patient's lifestyle, which slows down or even stops the development of the disease.

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The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!