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

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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 < 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.

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Acknowledgements

The authors are grateful to all their colleagues who participated in the J-RBR. This study was supported in part by the committee of the Japanese Society of Nephrology and a grant-in-aid for Intractable Renal Diseases Research, Research on Rare and Intractable Diseases, and Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan. We would like to thank Editage (www.editage.com) for English language editing.

Author information

Authors and Affiliations

  1. Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazaki, Nagakute, 480-1195, Japan

    Takayuki Katsuno & Yasuhiko Ito

  2. Department of Pediatrics, Tokushima University Graduate School of Medicine, Tokushima, Japan

    Shoji Kagami

  3. Department of Pathology, National Hospital Organization Chibahigashi National Hospital, Chiba, Japan

    Hiroshi Kitamura

  4. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Shoichi Maruyama

  5. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan

    Akira Shimizu

  6. Department of Human Resource Development of Dialysis Therapy for Kidney Disease, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan

    Hitoshi Sugiyama

  7. Department of Internal Medicine, Sendai Hospital of East Japan Railway Company, Sendai, Japan

    Hiroshi Sato

  8. Department of Nephrology, Kanazawa Medical University, Uchinada, Japan

    Hitoshi Yokoyama

  9. Department of Nephrology and Hypertension, Kawasaki Medical School, Kurashiki, Japan

    Naoki Kashihara

Authors
  1. Takayuki Katsuno
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  2. Yasuhiko Ito
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  3. Shoji Kagami
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  4. Hiroshi Kitamura
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  7. Hitoshi Sugiyama
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  8. Hiroshi Sato
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Corresponding author

Correspondence to Takayuki Katsuno.

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The authors have no conflicts of interest to declare.

Ethical standards

The ethical committee of the Japanese Society of Nephrology comprehensively examined and approved the study protocol.

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Informed consent was obtained from all participants included in this study.

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Katsuno, T., Ito, Y., Kagami, S. et al. A nationwide cross-sectional analysis of thrombotic microangiopathy in the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol 24, 789–797 (2020). https://doi.org/10.1007/s10157-020-01896-7

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  • DOI: https://doi.org/10.1007/s10157-020-01896-7

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