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Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey |
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| Authors: | Kunihiro Yamagata Joichi Usui Hitoshi Sugiyama Kosaku Nitta Takashi Wada Eri Muso Yoshihiro Arimura Akio Koyama Hirofumi Makino Seiichi Matsuo |
| Institution: | 1. Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan 2. Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan 3. Department of Medicine, Kidney Center, Tokyo Women’s Medical University, Tokyo, Japan 4. Division of Nephrology, Kanazawa University Hospital, Kanazawa, Japan 5. Department of Nephrology and Dialysis, The Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan 6. First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan 7. Department of Nephrology, Tsukuba Memorial Hospital, Tsukuba, Ibaraki, Japan 8. Department of Nephrology, Internal Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan |
| Abstract: | Renal involvement with significant organ damage is common in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). As a result, it is independently referred to ANCA-associated renal vasculitis. Clinically, ANCA-associated renal vasculitis is characterized by rapidly progressive glomerulonephritis. Pathologically, it is defined by pauci-immune type necrotizing and crescentic glomerulonephritis. According to previous reports from all over the world, the etiology, prevalence, and prognosis of RPGN including ANCA-associated renal vasculitis varies among races and periods. To elucidate the clinical characteristics of Japanese RPGN patients, a registry derived from a questionnaire survey was established in 1999 and maintained until 2006. As a result, 1,772 cases were collected, analyzed, and reported previously. In this mini-review, we outline the characteristic clinical findings of Japanese patients (Asian) with ANCA-associated renal vasculitis, based on the registry data. |
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