[Systemic sclerosis. A rare connective tissue disease with manifestations in many organs]

Systemic sclerosis (scleroderma) is a rare connective tissue disease which can affect most human organs. Systemic sclerosis is divided into two groups: one diffuse scleroderma form, which is often more serious, with extensive organ involvement, and a limited scleroderma form with good prognosis. During a period of 20 years from 1974 to 1994, 14 patients were diagnosed as having systemic sclerosis in a population of approximately 150,000. Five were classified as having the diffuse form, and nine as having the limited form. Ten patients were found to have antinuclear antibodies. All patients had Raynaud's phenomena and scleroderma. Involvement of the gastrointestinal tract/lungs and joint-tendon sheets were found in six and eight patients respectively. Muscles, heart and kidneys were involved in three of the patients. Our results correspond with those described in the literature.