title: A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan.
abstract: OBJECTIVE
  We conducted a cross-sectional nationwide survey to determine eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) prevalence and clinical features in Japan.
  
  
  METHODS
  Data for EGPA patients in 2008 were collected from 1,564 hospitals. In total, 965 patients were reported from 365 departments. In a second survey, clinical data for 473 patients were obtained.
  
  
  RESULTS
  We estimated that 1,866 (95% CI: 1,640-2,092) patients have EGPA in Japan (prevalence, 17.8/1,000,000). Of the 473 patients in the second survey, 315 fulfilled American College of Rheumatology (ACR) criteria or Lanham's criteria for EGPA. The mean age (± SD) of the 315 at onset was 55 ± 14 years, male to female ratio 1:2. 93% of patients had neurological manifestations, which were the organ system most frequently involved. Among 277 patients tested for myeloperoxidase (MPO)-/p anti-neutrophil cytoplasmic antibody (ANCA), 139 (50%) were positive, while only 6 of 238 were positive for proteinase3 (PR3)-/cANCA. MPO-ANCA-positive patients had renal involvement, mucous membrane or ophthalmological symptoms, and ENT symptoms more frequently, whereas cutaneous lesions and cardiovascular involvement were less common.
  
  
  CONCLUSIONS
  The prevalence of EGPA and the frequency of MPO-/p-ANCA-positivity in Japanese EGPA patients were mostly similar to those of Western countries. However, female predominance and a high frequency of neurological manifestations characterized Japanese patients.
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences , Okayama , Japan.
authors.forename: Ken-Ei
authors.initials: KE
authors.lastname: Sada
authors.forename: Koichi
authors.initials: K
authors.lastname: Amano
authors.forename: Ritei
authors.initials: R
authors.lastname: Uehara
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamamura
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D003430:Cross-Sectional Studies; D005260:Female; D006306:Health Surveys; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Anti-neutrophil cytoplasmic antibodies; Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA); Epidemiology; Vasculitis