SYSTEMIC SCLERODERMA - PROGNOSIS DETERMINED BY INTERNAL MANIFESTATIONS

Prognosis of systemic sclerosis (scleroderma, Ssc) is largely dependin g on involvement of internal organs. Abnormalities of the gastrointest inal tract are found most frequently (85%), especially decreased motil ity of the oesophagus,which has little impact on the longterm clinical course of Ssc. Pulmonary manifestations can be demonstrated in 40-90% of patients; one must distinguish between pulmonary hypertension or f ibrotic lung disease. The heart is affected in 50% of cases. Patchy or diffuse myocardial fibrosis, as well as pericarditis and pericardial effusions can induce symptoms of arrhythmia or congestive heart failur e. Renal involvement is associated with increased mortality and occurs in 45% of Ssc, producing proteinuria, hypertension, scleroderma renal crisis and renal failure. In conclusion, involvement of the lungs, he art and kidneys are determining factors for the longterm course of sys temic sclerosis.