Scleroderma - a disease that affects the connective tissue of the body, at which the seal (hardening) of the skin and blood vessels. As a rule, the disease process affects the whole body, and then talk about systemic scleroderma. Rare cases when changes are a small area of tissue, called localized scleroderma.
The disease affects people of all ages, including children. Women suffer systemic scleroderma three times more often than men. This is a fairly rare disease, occurring in 3 of every 100 000 people.
The causes of systemic sclerosis
Origin and causes of systemic scleroderma is unknown. Currently, many doctors tend to think of the disease manifestations of viral disease, triggering an autoimmune reaction, as in the tissues of people with systemic scleroderma, found a large number of virus particles in the blood and an increased amount of antibodies to them. What matters is a genetic predisposition, is more common in people from the same family. Of note the following contributing factors:
- Disorders of the nervous and endocrine systems;
- Transferred stress;
- Taking certain drugs (cytostatics);
- Prolonged contact with certain chemicals (silica dust, vinyl chloride, coal dust, some organic solvents).
Symptoms of systemic sclerosis
Systemic scleroderma refers to polisindromnym diseases as a result of the destruction of the blood vessels are affected, without exception, all the organs and tissues of the body. The most characteristic skin manifestations: first, a dense swelling of the skin, which is more condensed (sclerosing), then at this point the skin atrophy. Such phenomena occur throughout the body, but are most prominent on the face and extremities. In the affected areas of hair fall out, the skin is discolored, the limbs are deformed nail plates, at their fingertips painful ulcers appear, turning into ulcers and then scars. A person becomes immobile, mask-like appearance, difficult mouth opening. The skin is dry, dense, "mummified".
The lesions of the musculoskeletal system are expressed in myositis (muscle inflammation) caused by the replacement of normal muscle and tendon tissue fibrosis that leads to contractures. Joints are also limited in his movements, appears Syndrome morning stiffness, joint deformation fabric, there is pain, there is deposition of calcium salts. One of the characteristic features of systemic scleroderma is osteolysis terminal phalanges, as a result of which the fingers are shorter.
Characteristic of the symptoms of systemic scleroderma Raynaud's syndrome: a spastic attack, which resulted in the fingers or toes are cold, numb, in them a feeling of tingling, and they become white or bluish color. This happens under stress or temperature influence and can last from a few minutes to several hours. After the attack, bluish skin is red, there is a burning sensation and pain.
The defeat of the internal organs in systemic sclerosis manifests as a total destruction of all systems of circulatory disorders. Developing heart disease (cardiosclerosis, myocarditis, endocarditis), kidney damage (chronic nephropathy), gastrointestinal tract (esophagus stenosis is typical), etc. Also suffer nervous system and mental condition of the person.
The nature of the course and prognosis of systemic sclerosis
Systemic scleroderma can occur as acute, subacute, and chronic disease.
- During the most acute malignant, characterized by the rapid development of diseases of the internal organs and the constant increase of symptoms. In this case, systemic sclerosis prognosis is poor since a year after the onset of the disease comes a critical disruption of the internal organs.
- Subacute. Quickly developing skin lesions, muscles and joints, slow fibrosis of internal organs. Signs are growing steadily, but slowly. In this case, systemic scleroderma moderately favorable prognosis depends on the degree of generalization and process activity.
- Chronic. In the case of chronic course of systemic sclerosis the most favorable prognosis, because the symptoms are growing very slowly over years and decades, and organs for a long time do not lose their functions.
In addition to the course, for the prediction of systemic sclerosis has a value of clinical disease and the general condition of the patient.
Diagnosis of systemic sclerosis
Diagnosis of systemic scleroderma is carried out on the basis of characteristic clinical picture and the presence of diagnostic criteria - peculiar to the disease manifestations. For the diagnosis of systemic scleroderma is necessary to have a large number of small and diagnostic features. According to the system developed by the American Association of Rheumatology, systemic sclerosis diagnostic criteria are as follows:
- Great (main): scleroderma skin lesion with characteristic torso described above (the symptoms of systemic scleroderma) signs.
- Small (optional): characteristic of the affected finger (sclerodactyly), symmetrical basal pulmonary fibrosis (fibrotic lung), scarring on the fingertips, Raynaud's syndrome.
Also, for the diagnosis of systemic sclerosis conduct laboratory testing of blood, urine, immunodiagnostics, X-rays of the bones and joints, ultrasound of internal organs Heart conditions' diagnosis, that is, the full range of examinations of the bodies from which there is the most striking manifestation.
Treatment of systemic sclerosis
Treatment of systemic sclerosis is mainly symptomatic, aimed at alleviating the condition of the patient and a reduction in disease activity. In acute and subacute for the treatment of systemic sclerosis are used high doses of hormonal anti-inflammatory drugs. In all other cases, use the bracing means, vitamin therapy, immunocorrection. To attenuate fibrosis using drugs, softening the connective tissue (lidazy). It is also used drugs that enhance the work of the microvasculature.
Of great importance is physical therapy and physiotherapy. Physiotherapy used as a tonic and stimulating metabolism in the damaged tissues, which prevents them from atrophy. With the same purpose and used a specially designed physical therapy exercises. Treatment of systemic sclerosis should be continuous, restorative and developing musculoskeletal system activities should become a way of life for the patient, which provides slow down or even stop the development of the disease.
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