Scleroderma (persistent systemic sclerosis)

Autoimmune disease, principally of older women, and characterized by gradual increase of connective tissue in the skin, followed by hardening (sclerosis). A similar process can also occur in internal organs – in the oesophagus, for example. The condition is related to diseases such as polymyositis and dermatomyositis, and can also occur in association with them. All these disorders can be associated with arthritis. There is a benign form, scleroderma, which is restricted to the skin, causing patchy inflammation, hard, slippery and pale grey in the centre. In half of cases the disorder spreads further and develops into the severe form, affecting internal organs and causing more serious skin conditions. Changes in pigmentation occur, and calcium is deposited in the skin. Blood vessels are affected, causing circulatory problems in the fingertips which can cause them to die. Constriction of the oesophagus leads to difficulty in swallowing, and when the lungs are also affected there is a danger of respiratory difficulties and pneumonia. The resultant arthritis is similar to chronic rheumatoid arthritis. Treatment is directed in the first place at the prevention of serious complications. Drugs to dilate blood vessels can help to prevent the death of fingertips and toes; corticosteriods and anti- rheumatic drugs may also be prescribed.

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