NORMOTENSİVE RENAL CRISIS IN SCLERODERMA OVERLAPPED DERMATOMYOSITIS

Dermatomyositis (DM) is the nonsuppurative inflammation of the skeletal muscle with dermal rashes. One third of the cases have an overlapping connective tissue disease or malignancy may be accompanying in 10% of them. Scleroderma (SCL); is a connective tissue disease of unknown aetiology, may be seen alone or with overlap syndromes, characterized by systemic fibrosis. A serious and lifethreatening complication of SCL is "Scleroderma Renal Crisis" which is characterized by acceleratedmalignant hypertension. Normotensive renal crisis may be seen in glucocorticoid-treated SCL patients. Normotensive SCL renal crisis is a syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, pulmonary hemorrhage, recent prior corticosteroid use and poor outcome. Here we report a case of 22-year old scleroderma-overlapped dermatomyositis whose diagnosis established on Bohan-Peter criteria and treated with corticosteroids. She developed acute renal failure (ARF) in the fifth week of the treatment. She was given high dose captopril after the diagnosis of SCL kidney established by renal biopsy. Although she was maintained on haemodialysis five times, she had a fatal outcome on the fourteenth day of normotensive ARF.