anti-neutrophil cytoplasmic antibody, myeloperoxidase, microscopic polyangiitis, cyclophosphamide, corticosteroid

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1 Online publication June 24, 2009 ANCA JMAAV 1 2 ANCA JMAAV MPO-ANCA J Jpn Coll Angiol, 2009, 49: anti-neutrophil cytoplasmic antibody, myeloperoxidase, microscopic polyangiitis, cyclophosphamide, corticosteroid pauci-immune microscopic polyangiitis MPA Wegener Wegener s granulomatosis WG allergic granulomatous angiitis AGA 3 antineutrophil cytoplasmic antibody ANCA ANCA 3 proteinase 3 PR3 myeloperoxidase MPO PR3-ANCA MPO-ANCA PR3-ANCA WG MPO-ANCA MPA AGA ANCA MPA ,000 WG ,300AGA WG ANCA MPA WG 3 ANCA WG MPA PR3-ANCA WG 2 90 CYC 1 randomized controlled trial RCT 2 4 CYCAZAREM Cyclophosphamide versus Azathioprine as Remission Maintenance Therapy for ANCA-Associated Vasculitis RCT CYC (AZA) WGET Wegener s Granulomatosis Etanercept Trial WG tumor necrosis factor THE JOURNAL of JAPANESE COLLEGE of ANGIOLOGY Vol. 49,

2 ANCA JMAAV RCT 6 3 NORAM Non- Renal Vasculitis Alternative Treatment with Methotrexate unblinded prospective RCT CYC MTX ANCA 6 MTX 90 CYC MTX 70 CYC 47 4 RCT430 ANCA WG 364 MPA 66 WG MPA ANCA ANCA CYC ANCA 5 MPO-ANCA Prospective study of the severity-based treatment protocol for Japanese patients with MPO-ANCA-associated vasculitis JMAAV UMIN ID JMAAV 7 MPO-ANCA MPO-ANCA Birmingham Vasculitis Activity Score BVAS -2 Vasculitis Damage Index VDI SF36 MPO-ANCA RCT JMAAV 1 MPO-ANCA Table 1 Table 2 Table 3 54 Vol. 49, 2009

3 1 Table 1 I II. III. MPO-ANCA MPO-ANCA 1998 Table 2 I II. III Table CT Vol. 49,

4 ANCA JMAAV ,000/mm 3 120,000/mm 3 PaO 2 60 Torr 5 CYC 10g 1 3 RPGN 2 3 RPGN 1 Table Table 4 CYC mg IVCY g m 2 ST 2g 2 1g 5,000 10, mg 2 Table 4 A D PSL10 5mg CYC 6 AZA 1 BVAS CRP BVAS CYC 56 Vol. 49, 2009

5 1 A Table 4 PSL mg kg 40 60mg M-PSL g 3 M-PSL PSLPSL PSL40 60mg 1 2 PSL20mg IVCY g m 2 CYC mg kg mg Cr 1.8mg/dl 60IVCY CYC IVCY IVCY IVCY 2 WBC 3500 l CYC 3 6 CYC AZA mg kg mg 6 RPGN RPGN B RPGN Table 5 I IICYC PSL I II PSL mg kg III IV CYC III IV M-PSL500 1,000mg 3 PSL mg kg CYC25mg C IVCY CYC PSL D PSL mg kg 15 30mg CYC AZA mg kg mg 3 A 30 30PaO 2 60 Torr B rapidly progressive glomerulonephritis: RPGN CrCcr 50 MPO- ANCA Cr Ccr Cr 1 2 C D E F G ENT Vol. 49,

6 ANCA JMAAV Table 5 A CRP mg/dl mg/dl B Grade I Grade II Grade III Grade IV BVAS-2 VDI Table 5 QOL SF-36 v2 1 2 A BVAS-2 6 VDI B C 6 SF-36 v2 3 A MPO-ANCA B PSL MPO-ANCA Vol. 49, 2009

7 1 Table 6 JMAAV Web MPO-ANCA 17 Table RPGN MPO-ANCA Vol. 49,

8 ANCA JMAAV MPO-ANCA BVAS ANCA ANCA 1 Hoffman GS, Kerr GS, Leavitt RY et al: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med, 1992, 116: Jayne D, Rasmussen N, Andrassy K et al: A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med, 2003, 349: Wegener s Granulomatosis Etanercept Trial (WGET) Research Group: Etanercept plus standard therapy for Wegener s granulomatosis. N Engl J Med, 2005, 352: De Groot K, Rasmussen N, Bacon PA et al: Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum, 2005, 52: MPO-ANCA (JMAAV) Vol. 49, 2009

9 1 Prospective Study of ANCA-associated Vasculitis: JMAAV Shoichi Ozaki 1 and Kimimasa Nakabayashi 2 1 Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa, Japan 2 1st Department of Internal Medicine, Kyorin University, Tokyo, Japan anti-neutrophil cytoplasmic antibody, myeloperoxidase, microscopic polyangiitis, cyclophosphamide, corticosteroid Among small-vessel vasculitides, microscopic polyangiitis (MPA), Wegener s granulomatosis (WG), and allergic granulomatous angiitis (AGA) are known collectively as ANCA-associated vasculitis (AAV) because of the involvement of antineutrophil cytoplasmic antibodies (ANCA) as the common pathogenesis. Major target antigens of ANCA associated with vasculitis are myeloperoxidase (MPO) and proteinase 3 (PR3). MPO-ANCA-associated vasculitis is more frequent in Japan, whereas PR3-ANCA-associated vasculitis is more common in Europe and the United States. To establish evidence for Japanese patients with MPO-ANCA-associated vasculitis, the standard protocols for MPA have been evaluated in a prospective, open-labeled, multi-center study, called a JMAAV study. In this trial, patients with newly diagnosed MPA were stratified into those with the severe form, most severe form, or mild limited form of the disease. Patients received the regimen according to the therapy protocol, and were followed up for 18 months. The primary end point was induction of remission, and the rates of severe adverse effects, including death and end-stage renal failure, were also evaluated. The enrollment of patients was completed at the end of September 2006, and 50 patients were enrolled. The follow-up of all patients was completed at the end of March 2008, and an intensive analysis is currently underway. (J Jpn Coll Angiol, 2009, 49: 53 61) Vol. 49, 2009 Online publication June 24,