Comparison of phenotype and outcome in microscopic polyangiitis between Europe and Japan

Shunsuke Furuta, Afzal N. Chaudhry, Yoshitomo Hamano, Shouichi Fujimoto, Hiroko Nagafuchi, Hirofumi Makino, Seiichi Matsuo, Shoichi Ozaki, Tomomi Endo, Eri Muso, Chiharu Ito, Eiji Kusano, Mieko Yamagata, Kei Ikeda, Daisuke Kashiwakuma, Itsuo Iwamoto, Kerstin Westman, David Jayne

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72 Citations (Scopus)


Objective. There are differences between Europe and Japan in the incidence and antineutrophil cytoplasmic antibody (ANCA) serotype of patients with microscopic polyangiitis (MPA). However, differences in phenotype or outcome have not been explored. We aimed to identify differences in phenotype and outcome of MPA between Europe and Japan. Methods. Sequential cohorts of patients with MPA and renal limited vasculitis were collected from European and Japanese centers (n = 147 and n = 312, respectively). Trial databases from the European Vasculitis Society and the Japanese patients with Myeloperoxidase (MPO)-ANCA-Associated Vasculitis (JMAAV) trial were studied (n = 254 and n = 48, respectively). We evaluated baseline characteristics including ANCA status and organ involvement, treatment, survival, and renal survival. Differences in survival and renal survival were studied using multivariate analysis. Results. The non-trial cohorts showed patients with MPA in Japan had a higher age at onset, more frequent MPO-ANCA positivity, lower serum creatinine, and more frequent interstitial pneumonitis than those in Europe (all p < 0.01). Comparisons between the trial databases demonstrated similar results. Cumulative patient survival and renal survival rates were not different between Europe and Japan (p = 0.71 and p = 0.38, respectively). Multivariate analysis identified age at onset, serum creatinine, gastrointestinal, and respiratory involvement as factors with higher risk of death. For endstage renal failure, serum creatinine and use of plasma exchange were identified as factors with higher risk, and immunosuppressant use as lower risk factors. Conclusion. Phenotypes in patients with MPA were different between Europe and Japan. However, the outcomes of patient survival and renal survival were similar. (First Release Jan 15 2014; J Rheumatol 2014;41:325-33; doi:10.3899/jrheum.130602).

Original languageEnglish
Pages (from-to)325-333
Number of pages9
JournalJournal of Rheumatology
Issue number2
Publication statusPublished - Feb 2014


  • ANCA-associated vasculitis
  • Europe
  • Japan
  • Microscopic polyangiitis
  • Outcome
  • Phenotype

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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