• Masahiro Okabe, Arisa Kobayashi, Hirokazu Marumoto, Kentaro Koike, Izumi Yamamoto, Tetsuya Kawamura, Nobuo Tsuboi, and Takashi Yokoo.
• Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Japan.
• Intern. Med. 2021 Mar 15; 60 (6): 917-922.

AbstractPatients with atypical hemolytic uremic syndrome (aHUS) associated with a C3 p.Ile1157Thr mutation show a relatively high renal survival and low mortality rates, but renal histopathological findings after recurrence have been rarely reported. A 30-year-old man with a C3 p.Ile1157Thr mutation experienced a third recurrence of thrombotic microangiopathies with neurological and gastrointestinal disorders. A renal biopsy performed during the recovery phase of acute kidney injury revealed collapsed glomeruli and arteriolar vacuolization. Approximately 10% of glomeruli were globally sclerotic, despite the absence of arterio-/arteriolo-sclerosis. These findings suggest substantial progression of irreversible injuries in multiple organs, including kidneys, which occurs in aHUS patients with repeated thrombotic microangiopathies.

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