Vol.22 No.3

Original Article

Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study

Authors

Shoichi Ozaki1 , Tatsuya Atsumi2 , Taichi Hayashi3 , Akihiro Ishizu4 , Shigeto Kobayashi5 , Shunichi Kumagai6 , Yasuyuki Kurihara7 , Manae S. Kurokawa8 , Hirofumi Makino9 , Hiroko Nagafuchi1 , Kimimasa Nakabayashi10 , Norihiro Nishimoto11, Machi Suka12, Yasuhiko Tomino13, Hidehiro Yamada 1, Kunihiro Yamagata14, Masaharu Yoshida15, Wako Yumura16

  • Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, 216-8511, Japan
  • Department of Medicine II, Clinical Immunology and Metabolism, Hokkaido University, Sapporo, Japan
  • Division of Clinical Immunology, Graduate School of Comprehensive Human Science, University of Tsukuba, Tsukuba, Japan
  • Faculty of Health Science, Hokkaido University, Sapporo, Japan
  • Division of Rheumatology, Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan
  • Department of Clinical Pathology and Immunology, Kobe University Graduate School of Medicine, Kobe, Japan
  • Department of Radiology, St. Marianna University School of Medicine, Kawasaki, Japan
  • Division of Clinical Proteomics and Molecular Medicine, St. Marianna University Graduate School of Medicine, Kawasaki, Japan
  • Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan
  • The First Department of Internal Medicine, Kyorin University School of Medicine, Mitaka, Japan
  • Laboratory of Immune Regulation, Wakayama Medical University, Wakayama, Japan
  • Department of Public Health and Environmental Medicine, The Jikei University School of Medicine, Tokyo, Japan
  • Division of Nephrology, Department of Internal Medicine, Juntendo University, Tokyo, Japan
  • Department of Nephrology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan
  • Department of Nephrology, Tokyo Medical University Hachioji Medical Center, Hachioji, Japan
  • Department of Nephrology, Jichi Medical University, Shimotsuke, Japan
Received:

4 July 2011

Accepted:

22 August 2011

Published online:

18 September 2011

Full Text

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Abstract

We (JMAAV [Japanese patients with MPOANCA-associated vasculitis] Study Group) performed a prospective, open-label, multi-center trial to evaluate the usefulness of severity-based treatment in Japanese patients with myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA)-associated vasculitis. Patients with MPO-ANCA-associated vasculitis received a severitybased regimen according to the appropriate protocol: lowdose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; and the severe-form regimen plus plasmapheresis in those with the most severe form. We followed up the patients for 18 months. The primary end points were the induction of remission, death, and end-stage renal disease (ESRD). Fifty-two patients were registered, and 48 patients were enrolled in this study (mild form, n = 23; severe form, n = 23; most severe form, n = 2). Among the 47 patients who received the predefined therapies, 42 achieved remission within 6 months, 5 died, and 1 developed ESRD. Disease flared up in 8 of the 42 patients with remission during the 18-month follow-up period. The JMAAV trial is the first prospective trial for MPO-ANCA-associated vasculitis to be performed in Japan. The remission and death rates were comparable to those in several previous clinical trials performed in western counties. The regimen employed in this trial was tailor-made based on patients’ disease severity and disease type, and it seems that standardization can be consistent with treatment choices made according to severity.

Key words

Anti-neutrophil cytoplasmic antibody - Microscopic polyangiitis - Prophylaxis - Pulmonary-limited vasculitis - Severity-based treatment