title: Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study.
abstract: We (JMAAV [Japanese patients with MPO-ANCA-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 severity-based regimen according to the appropriate protocol: low-dose 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.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki 216-8511, Japan. ozk@marianna-u.ac.jp
authors.forename: Shoichi
authors.initials: S
authors.lastname: Ozaki
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Atsumi
authors.forename: Taichi
authors.initials: T
authors.lastname: Hayashi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Ishizu
authors.forename: Shigeto
authors.initials: S
authors.lastname: Kobayashi
authors.forename: Shunichi
authors.initials: S
authors.lastname: Kumagai
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Kurihara
authors.forename: Manae S
authors.initials: MS
authors.lastname: Kurokawa
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
authors.forename: Hiroko
authors.initials: H
authors.lastname: Nagafuchi
authors.forename: Kimimasa
authors.initials: K
authors.lastname: Nakabayashi
authors.forename: Norihiro
authors.initials: N
authors.lastname: Nishimoto
authors.forename: Machi
authors.initials: M
authors.lastname: Suka
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Tomino
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Yamada
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
authors.forename: Masaharu
authors.initials: M
authors.lastname: Yoshida
authors.forename: Wako
authors.initials: W
authors.lastname: Yumura
authors.forename: K
authors.initials: K
authors.lastname: Amano
authors.forename: Y
authors.initials: Y
authors.lastname: Arimura
authors.forename: K
authors.initials: K
authors.lastname: Hatta
authors.forename: S
authors.initials: S
authors.lastname: Ito
authors.forename: H
authors.initials: H
authors.lastname: Kikuchi
authors.forename: E
authors.initials: E
authors.lastname: Muso
authors.forename: H
authors.initials: H
authors.lastname: Nakashima
authors.forename: Y
authors.initials: Y
authors.lastname: Ohsone
authors.forename: Y
authors.initials: Y
authors.lastname: Suzuki
authors.forename: H
authors.initials: H
authors.lastname: Hashimoto
authors.forename: A
authors.initials: A
authors.lastname: Koyama
authors.forename: S
authors.initials: S
authors.lastname: Matsuo
authors.forename: H
authors.initials: H
authors.lastname: Kato
mesh_terms: D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy* / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D044466:Asian People; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan; D008297:Male; D008875:Middle Aged; D009195:Peroxidase / Q000276:immunology*; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D009195:Peroxidase