Scleroderma: what is it?

A chronic disease whose causes remain unknown, scleroderma is manifested by the presence of specific autoantibodies, diffuse fibrosis, vascular damage to the skin but which can extend to the organs (digestive system, heart, kidneys). More common in women than in men, it usually occurs between the ages of 20 and 50 but can affect children.

What are the symptoms of scleroderma?

In the localized form, the skin is thickened and presents lesions of fibrosis in plates or strips. Limited systemic sclerosis (syndrome Crest) presents a slowly progressive form with thickening of the skin of the face, feet, hands, forearms, legs which may be accompanied by gastroesophageal reflux disease. Painful digital ulcers and seizures‘arthritis significantly affect the quality of life of patients. In the fingers and toes, Raynaud’s phenomenon (sensation of numbness, change in color of the skin) is considered a harbinger of pathology. In its diffuse form (generalized systemic sclerosis with diffuse cutaneous involvement), scleroderma can affect the joints (retraction) and the digestive system. Rapidly evolving, this type can present major complications in the lungs (interstitial lung disease) or kidneys (crisis renal scleroderma).

Causes and treatment of scleroderma

For reasons still unclear, the endothelial cells lining the interior of the small vessels of the skin and organs are destroyed leading to an inflammatory reaction and the production ofautoantibodies specific. This apoptosis cell leads to the excessive formation of fibroblasts which usually participate in tissue repair with overproduction of collagen causing fibrosis. Scleroderma is therefore considered an autoimmune disease in which the immune system of the person turns against her.

The causes of the disease are unknown. Of the predispositions genetics are discussed. the diagnostic is based on the detection of circulating autoantibodies. There is no treatment curative Against scleroderma, patient management is based on therapies adapted to the organs affected. Some immunosuppressants can improve pulmonary complications.