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

Takayuki Katsuno, Yasuhiko Ito, Shoji Kagami, Hiroshi Kitamura, Shoichi Maruyama, Akira Shimizu, Hitoshi Sugiyama, Hiroshi Sato, Hitoshi Yokoyama, Naoki Kashihara

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


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 < 0.01). Conclusion: This is the first report of epidemiological and clinical data of biopsy-proven TMA in Japan. The characteristics of TMA with diversity based on the underlying disease and age group were reported.

Original languageEnglish
Pages (from-to)789-797
Number of pages9
JournalClinical and Experimental Nephrology
Issue number9
Publication statusPublished - Sep 1 2020


  • Connective tissue disease
  • Hemolytic uremic syndrome
  • Japan renal biopsy registry (J-RBR)
  • Renal biopsy
  • Thrombotic microangiopathy
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Physiology
  • Nephrology
  • Physiology (medical)


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