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.
저자들은 피부근염에 동반된 혈전혈소판감소자색반병을 진단하고 고농도의 스테로이드 투약과 혈장교환술로 치료하였으나 일시적인 호전 이후 재발하여 면역억제제인 rituximab을 투약하였고 피부근염과 혈전혈소판감소자색반병이 호전된 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
