HOSHINO Junichi    Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine    Position   Professor and Division head
Article types	Original article
Language	English
Peer review	Non peer reviewed
Title	A case of thrombotic microangiopathy associated with polymyositis
Journal	Formal name：Clinical nephrology Abbreviation：Clin Nephrol ISSN code：03010430/03010430
Domestic / Foregin	Foregin
Volume, Issue, Page	95(6),pp.339-344
Author and coauthor	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
Publication date	2021/06
Summary	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