Takayuki Katsuno1, Yasuhiko Ito2, Shoji Kagami3, Hiroshi Kitamura4, Shoichi Maruyama5, Akira Shimizu6, Hitoshi Sugiyama7, Hiroshi Sato8, Hitoshi Yokoyama9, Naoki Kashihara10. 1. Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazaki, Nagakute, 480-1195, Japan. t-katsuno@aichi-med-u.ac.jp. 2. Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazaki, Nagakute, 480-1195, Japan. 3. Department of Pediatrics, Tokushima University Graduate School of Medicine, Tokushima, Japan. 4. Department of Pathology, National Hospital Organization Chibahigashi National Hospital, Chiba, Japan. 5. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan. 6. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. 7. Department of Human Resource Development of Dialysis Therapy for Kidney Disease, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. 8. Department of Internal Medicine, Sendai Hospital of East Japan Railway Company, Sendai, Japan. 9. Department of Nephrology, Kanazawa Medical University, Uchinada, Japan. 10. Department of Nephrology and Hypertension, Kawasaki Medical School, Kurashiki, Japan.
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.
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.
Authors: Marja Kovala; Minna Seppälä; Kati Kaartinen; Seppo Meri; Eero Honkanen; Anne Räisänen-Sokolowski Journal: J Clin Med Date: 2022-05-31 Impact factor: 4.964