title: Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey.
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.
authors.affiliation: Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan. kidney@md.tsukuba.ac.jp.
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
authors.affiliation: Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
authors.forename: Joichi
authors.initials: J
authors.lastname: Usui
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sugiyama
authors.affiliation: Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.
authors.forename: Kosaku
authors.initials: K
authors.lastname: Nitta
authors.affiliation: Division of Nephrology, Kanazawa University Hospital, Kanazawa, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Wada
authors.affiliation: Department of Nephrology and Dialysis, The Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Muso
authors.affiliation: First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.affiliation: Department of Nephrology, Tsukuba Memorial Hospital, Tsukuba, Ibaraki, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Koyama
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
authors.affiliation: Department of Nephrology, Internal Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Matsuo
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000175:diagnosis / Q000208:ethnology* / Q000276:immunology / Q000628:therapy; D044466:Asian People; D005921:Glomerulonephritis / Q000175:diagnosis / Q000208:ethnology* / Q000276:immunology / Q000628:therapy; D006306:Health Surveys; D006801:Humans; D007564:Japan / Q000453:epidemiology; D011379:Prognosis; D011795:Surveys and Questionnaires; D013997:Time Factors