Onion Skin Lesion of the Renal Small Artery in a Patient With Polymyositis and Hemolytic Uremic Syndrome

Kurihara, Saburo; Sawa, Naoki; Inui, Keita; Ikuma, Daisuke; Oba, Yuki; Mizuno, Hiroki; Sekine, Akinari; Yamanouchi, Masayuki; Hasegawa, Eiko; Suwabe, Tatsuya; Kono, Kei; Kinowaki, Keiichi; Ohashi, Kenichi; Tomita, Shigeki; Okubo, Tadanobu; Ubara, Yoshifumi

doi:10.1016/j.xkme.2022.100539

Cited by 2 publications

(1 citation statement)

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“…There were no autoantibodies or skin manifestations, including Raynaud's symptoms, which are characteristic of scleroderma. Among other autoimmune diseases, we found that polymyositis also has renal involvement similar to that seen in scleroderma renal crisis after steroid administration ( 9 , 10 ). This suggests that steroid administration can cause lesions corresponding to scleroderma renal crisis in diseases other than scleroderma.…”

Section: Discussionsupporting

confidence: 54%

Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions

et al. 2023

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A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites developed. A lymph node biopsy indicated TAFRO syndrome. The patient's renal function deteriorated, and dialysis was started. Refractory hypertension and subsequent encephalopathy developed. Treatment was started with an anti-IL-6 receptor antibody and an anti-CD20 monoclonal antibody. A kidney biopsy showed malignant nephrosclerosis-like microangiopathy and glomerular collapse due to narrowing of the small arteries. The majority of TAFRO syndrome cases are adult-onset, with glomerular microangiopathy. To our knowledge, this is the first report of adolescent-onset TAFRO syndrome presenting with malignant nephrosclerosis-like lesions associated with hypertension.

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Section: Discussionsupporting

confidence: 54%