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  • Title: Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions.
    Author: Nakayama Y, Mizuno H, Sawa N, Suwabe T, Yamanouchi M, Ikuma D, Hasegawa E, Hoshino J, Sekine A, Oba Y, Kono K, Kinowaki K, Ohashi K, Suzuki K, Sato Y, Shimizu A, Yamaguchi Y, Ubara Y.
    Journal: Intern Med; 2023 Aug 01; 62(15):2223-2229. PubMed ID: 36517029.
    Abstract:
    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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