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A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity |
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| Authors: | Akio Koyama Kunihiro Yamagata Hirofumi Makino Yoshihiro Arimura Takashi Wada Kosaku Nitta Hiroshi Nihei Eri Muso Yoshio Taguma Hidekazu Shigematsu Hideto Sakai Yasuhiko Tomino Seiichi Matsuo |
| Institution: | 1. Ibaraki Prefuctural University, Ami, Japan 2. Department of Nephrology, Institute of Clinical Medicine, Graduate School of Comprehensive Human Sciences, University of Tsukuba, 1-1-1 Ten-oudai, Tsukuba, Ibaraki, 305-8575, Japan 3. Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan 4. First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan 5. Department of Laboratory Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan 6. Department of Medicine, Kidney Center, Tokyo Women’s Medical University, Tokyo, Japan 7. Department of Nephrology and Dialysis, The Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan 8. Department of Nephrology, Sendai Shakaihoken Hospital, Sendai, Japan 9. Department of Pathology, Shinsyu University, Matsumoto, Japan 10. Division of Nephrology and Metabolism, Department of Internal Medicine, Tokai University, School of Medicine, Kanagawa, Japan 11. Division of Nephrology, Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan 12. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan |
| Abstract: | BackgroundThe etiology, prevalence, and prognosis of rapidly progressive glomerulonephritis (RPGN) including renal vasculitis vary among races and periods.MethodTo improve the prognosis of Japanese RPGN patients, we conducted a nationwide survey of RPGN in the nephrology departments of 351 tertiary hospitals, and found 1772 patients with RPGN (Group A: diagnosed between 1989 and 1998, 884 cases; Group B: diagnosed between 1999 and 2001, 321 cases; and Group C: diagnosed between 2002 and 2007, 567 cases). ANCA subclasses, renal biopsy findings, treatment, outcome and cause of death were recorded.ResultThe most frequent primary disease was renal-limited vasculitis (RLV) (42.1%); the second was microscopic polyangiitis (MPA) (19.4%); the third was anti-GBM-associated RPGN (6.1%). MPO-ANCA was positive in 88.1% of RLV patients and 91.8% of MPA patients. The proportion of primary renal diseases of RPGN was constant during those periods. The most frequent cause of death was infectious complications. The serum creatinine at presentation and the initial dose of oral prednisolone decreased significantly in Groups B and C compared to Group A. However, both patient and renal survival rates improved significantly in Groups B and C (survival rate after six months in Group A: 79.2%, Group B: 80.1%, and Group C: 86.1%. Six-month renal survival in Group A: 73.3%, Group B: 81.3%, and Group C: 81.8%).ConclusionEarly diagnosis was the most important factor for improving the prognosis of RPGN patients. To avoid early death due to opportunistic infection in older patients, a milder immunosuppressive treatment such as an initial oral prednisolone dose reduction with or without immunosuppressant is recommended. |
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