Rituximab Treatment in a Case of Dermatomyositis Accompanied by Thrombotic Thrombocytopenic Purpura / 대한내과학회지

ABSTRACT

Thrombotic thrombocytopenic purpura (TTP) is an uncommon, serious disease known to be related with infection, toxin, pregnancy and autoimmune disease. Autoimmune disease-related TTP is rare, difficult to diagnose and can rapidly become fatal if left untreated. We report the case of a 59 year-old Korean female with TTP associated with dermatomyositis flare. The patient was suspected to have amyopathic dermatomyositis and was treated with moderate doses of oral glucocorticoids. One month later, muscle weakness developed with dermatomyositis flare, and the patient showed confusion, acute renal failure, thrombocytopenia and microangiopathic hemolytic anemia. She was diagnosed with TTP associated with dermatomyositis flare. After prompt plasmapheresis treatment with high dose glucocorticoid therapy, her confusion, thrombocytopenia and anemia were improved. However, oliguric renal failure and myopathy remained, and thrombocytopenia and anemia recurred. After starting additional rituximab treatment, the clinical manifestation of dermatomyositis and TTP improved markedly.