Clin Nephrol. 2021 Jun;95(6):339-344. doi: 10.5414/CN109989.
A 60-year-old Japanese woman with polymyositis (PM) developed hemolytic anemia (hemoglobin of 7.3 g/dL), thrombocytopenia (platelet of 9.1×10/µL), and acute kidney injury (Cre of 4.7 mg/dL) at 14 days after starting steroid therapy. Renal biopsy revealed glomerular endothelial swelling with fibrin thrombi and fragmented erythrocytes in the capillary lumens. Hemolytic uremic syndrome (HUS) with thrombotic microangiopathy (TMA) was diagnosed. Hemodialysis and plasma exchange/plasma transfusion were initiated, but HUS did not subside. After 45 days, the patient died of hemorrhagic respiratory failure. Autopsy showed fibrin thrombi filling the glomerular vascular pole and the small arteries in most glomeruli, resulting in glomerular collapse and glomerular basement membrane (GBM) duplication. Although renal involvement by PM is rare, HUS/TMA should be remembered as one of the serious renal complications of PM.
一位 60 岁的日本女性，患有多发性肌炎（PM），在开始类固醇治疗 14 天后出现溶血性贫血（血红蛋白 7.3g/dL）、血小板减少症（血小板 9.1×10/µL）和急性肾损伤（Cre 4.7mg/dL）。肾脏活检显示肾小球内皮肿胀，毛细血管腔内有纤维蛋白血栓和破碎的红细胞。诊断为伴有血栓性微血管病（TMA）的溶血性尿毒症综合征（HUS）。开始进行血液透析和血浆置换/血浆输注，但 HUS 并未缓解。45 天后，患者死于出血性呼吸衰竭。尸检显示纤维蛋白血栓充满肾小球血管极和大多数肾小球的小动脉，导致肾小球塌陷和肾小球基底膜（GBM）重复。尽管 PM 引起的肾脏受累很少见，但 HUS/TMA 应作为 PM 的严重肾脏并发症之一加以记住。
