ホシノ　ジユンイチ   Hoshino Jiyun'ichi   星野　純一    所属   医学部 医学科（東京女子医科大学病院）    職種   教授・基幹分野長
論文種別	原著
言語種別	英語
査読の有無	査読なし
表題	A case of thrombotic microangiopathy associated with polymyositis
掲載誌名	正式名：Clinical nephrology 略　 称：Clin Nephrol ISSNコード：03010430/03010430
掲載区分	国外
巻・号・頁	95(6),pp.339-344
著者・共著者	Fukuda Makoto, Mizuno Hiroki, Hiramatsu Rikako, Sekine Akinari, Kawada Masahiro, Hasegawa Eiko, Yamanouchi Masayuki, Suwabe Tatsuya, Hoshino Junichi, Sawa Naoki, Takaichi Kenmei, Kinowaki Keiichi, Ohashi Kenichi, Fujii Takeshi, Miyazono Motoaki, Ubara Yoshifumi
発行年月	2021/06
概要	A 60-year-old Japanese woman with polymyositis (PM) developed hemolytic anemia (hemoglobin of 7.3 g/dL), thrombocytopenia (platelet of 9.1×104/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.
DOI	10.5414/CN109989
PMID	33769275