A case of thrombotic microangiopathy associated with polymyositis
2021; Dustri-Verlag; Volume: 95; Issue: 6 Linguagem: Inglês
10.5414/cn109989
ISSN0301-0430
AutoresMakoto Fukuda, Hiroki Mizuno, Rikako Hiramatsu, Akinari Sekine, Masahiro Kawada, Eiko Hasegawa, Masayuki Yamanouchi, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Kenichi Ohashi, Takeshi Fujii, Motoaki Miyazono, Yoshifumi Ubara,
Tópico(s)Genetic Syndromes and Imprinting
ResumoA 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.
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