title: Pulmonary involvements of anti-neutrophil cytoplasmic autoantibody-associated renal vasculitis in Japan.
abstract: BACKGROUND
  Pulmonary involvement is one of the hallmark lesions of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) as well as rapidly progressive glomerulonephritis (RPGN). However, the pulmonary involvement of AAV patients seems to differ between Europe and Japan, as does the ANCA serotype.
  
  
  METHODS
  This retrospective and prospective multicenter cohort study collected the clinical data of the features and outcomes of 1772 RPGN patients treated from 1989 to 2007 in Japan. Based on this nationwide RPGN survey, we analyzed the cases of 1147 AAV patients.
  
  
  RESULTS
  We found that 52.3% of the AAV patients had pulmonary involvements: 15.4% of the AAV patients had alveolar hemorrhage (AH), 26.2% had interstitial lung disease (ILD), 2.8% had bronchial asthma, 2.4% had pulmonary granuloma and 12.8% had a chest X-ray abnormality without AH, ILD or pulmonary granuloma. Patient survival was significantly different among the following six groups: the 5-year survival rate was 41.5% in the patients with AH, 50.2% in those with ILD, 67.9% in those with bronchial asthma, 62.5% in those with pulmonary granuloma, 55.8% in those with chest X-ray abnormality and 73.3% in those without pulmonary involvement. AH was one of the predictors of 1- and 5-year mortality for patient survival in AAV, and ILD was added as one of the predictors of 5-year mortality.
  
  
  CONCLUSIONS
  In these AAV patients, not only AH but also ILD was frequently observed. AH was associated with the prognosis, but ILD was associated with the long-term prognosis of AAV.
authors.affiliation: Department of Nephrology, Tokyo Medical University Ibaraki Medical Center, Ami, Ibaraki, Japan.
authors.forename: Kouichi
authors.initials: K
authors.lastname: Hirayama
authors.affiliation: Department of Nephrology, Tokyo Medical University Ibaraki Medical Center, Ami, Ibaraki, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Kobayashi
authors.affiliation: Department of Nephrology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
authors.forename: Joichi
authors.initials: J
authors.lastname: Usui
authors.affiliation: First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Tokyo, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.affiliation: Department of Chronic Kidney Disease and Peritoneal Dialysis, 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, Shinjuku-ku, Tokyo, Japan.
authors.forename: Kosaku
authors.initials: K
authors.lastname: Nitta
authors.affiliation: Division of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Institute, Osaka, Japan.
authors.forename: Eri
authors.initials: E
authors.lastname: Muso
authors.affiliation: Department of Disease Control and Homeostasis, Kanazawa University, Kanazawa, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Wada
authors.affiliation: Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Matsuo
authors.affiliation: Department of Nephrology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
mesh_terms: D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D005060:Europe; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D006470:Hemorrhage / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007564:Japan; D008171:Lung Diseases / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate
keywords: ANCA-associated vasculitis; alveolar hemorrhage; interstitial lung disease; mortality