A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan

Mod Rheumatol. 2014 Jul;24(4):640-4. doi: 10.3109/14397595.2013.857582. Epub 2013 Dec 2.


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.

Conclusion: 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.

Keywords: Anti-neutrophil cytoplasmic antibodies; Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA); Epidemiology; Vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Churg-Strauss Syndrome / diagnosis*
  • Churg-Strauss Syndrome / drug therapy
  • Churg-Strauss Syndrome / epidemiology*
  • Cross-Sectional Studies
  • Female
  • Health Surveys
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Prevalence
  • Treatment Outcome


  • Immunosuppressive Agents