TY - JOUR
T1 - A nationwide cross-sectional analysis of thrombotic microangiopathy in the Japan Renal Biopsy Registry (J-RBR)
AU - Katsuno, Takayuki
AU - Ito, Yasuhiko
AU - Kagami, Shoji
AU - Kitamura, Hiroshi
AU - Maruyama, Shoichi
AU - Shimizu, Akira
AU - Sugiyama, Hitoshi
AU - Sato, Hiroshi
AU - Yokoyama, Hitoshi
AU - Kashihara, Naoki
N1 - Funding Information:
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.
Publisher Copyright:
© 2020, Japanese Society of Nephrology.
Copyright:
Copyright 2020 Elsevier B.V., All rights reserved.
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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.
AB - 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.
KW - Connective tissue disease
KW - Hemolytic uremic syndrome
KW - Japan renal biopsy registry (J-RBR)
KW - Renal biopsy
KW - Thrombotic microangiopathy
KW - Thrombotic thrombocytopenic purpura
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U2 - 10.1007/s10157-020-01896-7
DO - 10.1007/s10157-020-01896-7
M3 - Article
C2 - 32415379
AN - SCOPUS:85085031759
VL - 24
SP - 789
EP - 797
JO - Clinical and Experimental Nephrology
JF - Clinical and Experimental Nephrology
SN - 1342-1751
IS - 9
ER -