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

作者：Sada Ken Ei; Amano Koichi; Uehara Ritei; Yamamura Masahiro; Arimura Yoshihiro; Nakamura Yoshikazu; Makino Hirofumi^*

来源：Modern Rheumatology, 2014, 24(4): 640-644.

DOI：10.3109/14397595.2013.857582

• Anti-neutrophil cytoplasmic antibodies
• Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)
• Epidemiology
• Vasculitis

Objective. We conducted a cross-sectional nationwide survey to determine eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) prevalence and clinical features in Japan. %26lt;br%26gt;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. %26lt;br%26gt;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%26apos;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. %26lt;br%26gt;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.

• 出版日期2014-7