1. A case of thrombotic microangiopathy associated with polymyositis
- Author
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Rikako Hiramatsu, Masahiro Kawada, Akinari Sekine, Junichi Hoshino, Kenmei Takaichi, Keiichi Kinowaki, Tatsuya Suwabe, Yoshifumi Ubara, Makoto Fukuda, Takeshi Fujii, Naoki Sawa, Eiko Hasegawa, Motoaki Miyazono, Masayuki Yamanouchi, Hiroki Mizuno, and Kenichi Ohashi
- Subjects
Hemolytic anemia ,Anemia, Hemolytic ,Pathology ,medicine.medical_specialty ,Thrombotic microangiopathy ,medicine.medical_treatment ,Kidney ,urologic and male genital diseases ,Polymyositis ,Fibrin ,Renal Dialysis ,hemic and lymphatic diseases ,medicine ,Humans ,medicine.diagnostic_test ,biology ,Thrombotic Microangiopathies ,business.industry ,Glomerular basement membrane ,Acute kidney injury ,General Medicine ,Acute Kidney Injury ,Middle Aged ,medicine.disease ,female genital diseases and pregnancy complications ,medicine.anatomical_structure ,Nephrology ,Hemolytic-Uremic Syndrome ,biology.protein ,Female ,Renal biopsy ,Hemodialysis ,business - Abstract
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.
- Published
- 2021
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