title: Characteristics of granulomatosis with polyangiitis patients in Japan.
abstract: OBJECTIVE
  Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease with significant ethnic differences. Reports on characteristics of Japanese granulomatosis with polyangiitis (GPA) patients are limited, and this study was undertaken to determine the characteristics of Japanese GPA patients.
  
  
  METHODS
  This was a retrospective chart study of 24 Japanese GPA patients. GPA was defined according to the European Medicines Agency algorithm.
  
  
  RESULTS
  The percentage of MPO-ANCA-positive patients was 33.3%, higher than the percentages reported in studies from Western countries. MPO-ANCA-positive GPA patients differed from PR3-ANCA-positive GPA patients in organs involved at diagnosis with MPO-ANCA-positive patients having nose and sinus involvement less frequently compared to PR3-ANCA-positive patients. Interstitial lung infiltrates were more common among MPO-ANCA-positive GPA patients compared to PR3-ANCA-positive GPA patients.
  
  
  CONCLUSIONS
  Among Japanese GPA patients, the proportion of MPO-ANCA-positive patients is higher compared to reports from Western countries, and those patients are often different from the classical picture of GPA.
authors: Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo , Bunkyo-ku, Tokyo , Japan.
authors: Yumi
authors: Y
authors: Tsuchida
authors: Mihoko
authors: M
authors: Shibuya
authors: Hirofumi
authors: H
authors: Shoda
authors: Shuji
authors: S
authors: Sumitomo
authors: Kanae
authors: K
authors: Kubo
authors: Keigo
authors: K
authors: Setoguchi
authors: Keishi
authors: K
authors: Fujio
authors: Kazuhiko
authors: K
authors: Yamamoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D044466:Asian People; D005123:Eye / Q000276:immunology / Q000473:pathology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007564:Japan; D008168:Lung / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012867:Skin / Q000276:immunology / Q000473:pathology; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Anti-neutrophil cytoplasmic antibodies; Anti-neutrophil cytoplasmic antibody-associated vasculitis; Granulomatosis with polyangiitis; Wegener granulomatosis