Scleroderma belongs among the systemic autoimmune diseases and it affects many organs and tissues of the body. The disease occurs more frequently in women than in men.
The exact cause of the disease is unknown. The damage is caused by our own immune system, which starts to attack our own tissues. The reaction is mediated by the immune cells and antibodies directed against the tissues (so-called autoantibodies). In case of scleroderma, the immune reaction is directed mainly against small arterioles. These blood vessels are damaged, destroyed and replaced by the fibrous tissue. The inflammatory affection of blood vessels is referred to as the vasculitis.
The disease has multiple forms. The most typical form affects the skin and subcutaneous tissue; others may affect the whole organism. Skin manifestations include thickening of the skin, which is noticeable especially in fingers of the upper extremities. In severe forms, the fingers change into clumsy claws. The affected fingers often express symptoms of Raynaud's phenomenon (whitening of whole fingers or their parts). The face of the affected person may get a mask expression, also due to local deposition of fibrous tissue. In addition, the skin may be affected by occurrence of multiple violet spots all over the body.
Esophagus is typically affected internal organs. The deposits of connective tissue disrupt its motility and cause troubles with swallowing. Lung affection manifests with pulmonary fibrosis with gradually increasing shortness of breath and dry cough. The heart may be affected as well – the scleroderma decreases its pumping ability, causes arrhythmias and signs of heart failure. Kidney affection can be followed by chronic or acute kidney failure.
The diagnosis should be stated by a skilled rheumatologist according to patient's symptoms, physical examination and serological examination of blood that may confirm the presence of certain autoantibodies. There are special diagnostic criteria that the examination outcome must accomplish.
The basis of therapy includes regular administration of immunosuppressive drugs that dampen the immune reaction. There are many types of medications, probably the most known are the corticosteroids. The prognosis depends greatly on the exact type of the disease. The subtypes localized in the skin have significantly better prognosis than forms of scleroderma affecting the internal organs.