Patient characteristics at baseline.

Patient characteristics at baseline.</p

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

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.</p

Changing patient VAS score before and 6 months after MTX administration for each patient.

Changing patient VAS score before and 6 months after MTX administration for each patient.</p

Logistic regression analysis for the rate of achieving low disease activity based on the DAS28-CRP.

Logistic regression analysis for the rate of achieving low disease activity based on the DAS28-CRP.</p

Disease activity and medications after 6 months of MTX administration.

Disease activity and medications after 6 months of MTX administration.</p

Fig 1 -

ΔPEG (A) and patient VAS score (B) after 6 months of MTX administration in the anti-SSA antibody-positive and -negative groups. Horizontal lines represent the median, 1st quartile, and 3rd quartile. ΔPEG, numeric difference between patient VAS score and physician VAS score; VAS, visual analogue scale.</p

Patient characteristics at baseline between the patients who achieve LDA based on the DAS28-CRP and those that did not.

(DOCX)</p

Logistic regression analysis for the rate of achieving low disease activity according to the DAS28-CRP, including the methotrexate dose.

Logistic regression analysis for the rate of achieving low disease activity according to the DAS28-CRP, including the methotrexate dose.</p

Logistic regression analysis for rate of achieving low disease activity according to the DAS28-CRP, including baseline DAS28-CRP and disease duration.

Logistic regression analysis for rate of achieving low disease activity according to the DAS28-CRP, including baseline DAS28-CRP and disease duration.</p

The baseline characteristics and treatments of 111 patients.

The baseline characteristics and treatments of 111 patients.</p