A nationwide cross-sectional analysis of thrombotic microangiopathy in the Japan Renal Biopsy Registry (J-RBR)

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ORIGINAL ARTICLE

A nationwide cross‑sectional analysis of thrombotic microangiopathy in the Japan Renal Biopsy Registry (J‑RBR) Takayuki Katsuno1 · Yasuhiko Ito1 · Shoji Kagami2 · Hiroshi Kitamura3 · Shoichi Maruyama4 · Akira Shimizu5 · Hitoshi Sugiyama6 · Hiroshi Sato7 · Hitoshi Yokoyama8 · Naoki Kashihara9 Received: 25 December 2019 / Accepted: 23 April 2020 © Japanese Society of Nephrology 2020

Abstract Background  There have been only a few large-scale cohort studies that have reviewed accumulated cases of thrombotic microangiopathy (TMA). The aim of this study was to collect and analyze TMA cases based on the renal biopsy, as a nationwide survey in Japan. Methods  In this cross-sectional study, large nationwide data from the Japan Renal Biopsy Registry (J-RBR) were used. Among the patients registered in the J-RBR online system from July 2007 to July 2017, TMA cases were extracted and epidemiological data and clinical findings were investigated. Results  Out of the 38,495 patients enrolled in a period of 10 years, 152 (0.39%) cases had been diagnosed with TMA. The patient age was widely distributed, including 9.2%, 66.4%, and 24.3% for children, adults, and the elderly, respectively. There were various causes of TMA. Among them, hemolytic uremic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP) (16.4%), connective tissue disease (CTD)-related (17.1%), and drug-induced (16.4%) were frequently observed. The background factors of TMA were different in children and adults. In a comparison between groups consisting of HUS/TTP, CTD-related, and drug-induced, the HUS/TTP group was significantly younger (p = 0.01), and the drug-induced TMA group tended to have a high urinary protein positive rate (p = 0.05). A comparative analysis according to the age group showed significantly higher serum creatinine levels in the elderly (p