Systemic sclerosis

Systemic scleroderma is also called generalized, diffuse, progressive multiple sclerosis. The disease is among the group of collagen diseases with the defeat of all connective tissue structures in the body. The severity of the seizure and of the internal organs along with skin manifestations distinguishes this variant of scleroderma from focal.

The prevalence is up to 19 cases per 1000 000 population. Often ill women aged 30 to 50 years. In children the systemic form is very rare. Proven a significant increase in cases among the peoples of Africa and the Indians of North America.

According to the international statistical classification ICD-10 as a nosological unit included in the class M (a category of connective tissue diseases) and coded M34 "Systemic sclerosis".

The role of collagen in the development of the disease

The specific reason of the disease is unclear. Put forward various theories based on the detection of changes in the body of the sick person. Proven link the development of systemic sclerosis with excessive synthesis of collagen. Pathogenesis (pathological disorders and their progression) detects the excess collagen fibers in the walls of small arteries, muscles and stroma of organs.

System lesions in scleroderma is the accumulation of unwanted protein in the skin and all organs, where there is connective tissue, because the collagen is its major component, and up to 35% of the total protein composition.

The role of immunity in the pathogenesis of scleroderma

The second link, actively participating in the development of scleroderma, are immune cells. Proved changes of the interaction structures of the inner lining of blood vessels, smooth muscle, fibroblasts, lymphocytes T and b types, macrophages, eosinophils, mast cells.

Fibroblasts — the main "producers" of collagen, out of control and start to synthesize increased amounts. Anomaly aktiviziruyutsya:

1. platelet derived growth factor;
2. histamine;
3. transforming factor;
4. serotonin and thromboxane (in cold conditions);
5. nitrogenous compounds (with stress).

The American Association of rheumatology proposed in the diagnosis you consider "small" and "large" signs. By "small" they took:

• all the changes on the fingers (sclerodactyly);
• x-ray revealed fibrotic changes in the lungs in the absence of any other disease.

To big, decisive signs of the following: a typical seal, and change the skin on the fingers and in other places (face, torso, neck).

The Association believes that the correct diagnosis indicates one of the "big" symptom or at least two "small". Confirmed the specificity of diagnosis in 98% of cases.

Treatment of systemic sclerosis should be carried out, following main directions:

• the lifting of vascular spasm, blood circulation additional sleeper vessels;
• preventing the spread of fibrosis;
• the restoration of normal immune responses;
• treatment of internal injuries.

First of all, the patient is recommended to observe the regime: stop Smoking, avoid contact with poisonous substances, vibration to eliminate stress, nutrition to keen on spicy food, fried food, coffee.

In drug therapy in the treatment regimen included:

• vasodilators;
• medications, stopping the fibrosis of the tissues;
• anti-inflammatory medication, including corticosteroids;
• immunosuppressants.