ANCA 関連血管炎の診療ガイドライン２０１４年改訂版

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1 11981 : ANCA ANCA ANCA ANCA ANCA ANCA Chapel Hill ANCA ANCA : ANCA ANCA ANCA ANCAAntineutrophil cytoplasmic antibody ; ANCAassociated vasculitis ; AAV ANCA ANCA perinuclear ANCApANCA cytoplasmic ANCAcANCAp ANCA myeloperoxidasempo canca proteinase3pr3 ELISAEnzymeLinked ImmunoSorbent Assay MPOANCAPR3ANCA AAV microscopic polyangitis ; MPAgranulomatosis with polyangitis ; GPA eosinophilic granulomatosis with polyangitis ; EGPA renal limited vasculitis ; RLV ANCA AAV MPA GPA EGPA MPA polyarteritis nodosa ; PAN

2 11982 AAV 2016 MPAPAN GPA EGPA MPA PAN MPAPAN MPA AAV MPA MPA GPA AAV ANCA MPARLV GPAEGPA MPA PAN MPOANCA : MPOANCA PR3 ANCA MPOANCA PR3ANCA MPA rapidly progressive glomerulonephritis ; RPGN MPA GPA MPA MPA MPA RLV AAV pauciimmune RLV MPOANCA MPA RLV MPA GPA RLV MPA RLV RPGN RPGN GPA Wegener Wegener Chapel Hill PR3ANCA GPA PR3ANCA MPO ANCA GPA MPOANCA MPAEGPA GPA

3 AAV BVAS version 3 2kg 10RBC/HPFCr 14279mg/dLCr 28569mg/dL Cr57mg/dLCr Ccr EGPA Churg Strauss ChurgStrauss Chapel Hill : MPO ANCA EGPA AAV MPAGPAEGPA ACRAmerican College of RheumatologyCHCCChapel Hill Consensus ConferenceCHCCEMEAEuropean Medicines Agency CHCC ACR MPAGPAEGPA Birmingham vasculitis activity score ; BVAS BVAS BVAS version 3 BVAS BVAS

4 11984 AAV EUVAS AAV localized early systemic generalized severe refractory Cr 566mg/dL Cr 566mg/dL GC CY vasculitis damage index ; VDI VDI AAV EUVASEuropean Vasculitis Study Group BVAS prednisolone ; PSL AAV EULAREuropean League Against Rheumatismrecommendation EUVAS EULAR BSR British Society of Rheumatology EUVAS AAV glucocorticoid ; GCcyclophosphamide ; CY GC1mg/kg/day CY 2mg/ kg/day 15mg/kg methotrexate ; MTX GC PR3 ANCA GPA GPA MPA AZA MTX GC AAV GC BSR/BHPR British Health Professionals in Rheumatology CY PSL 30mg/day PSL 30mg/day GC rituximab AAV

5 AAV GPA MPA 375mg/m de novo ANCA ANCA ANCA ANCA otitis media with ANCA associated vasculitis ; OMAAV ANCA OMAAV GPA OMAAV ACR CHCCEMEA GPA OMAAV MPA GPA OMAAV MPOANCA OMAAV OMAAV GC AAV AAV MPA AAV GPA Jennette J, Falk R, Bacon P, et al : 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013 ; 65 : http ://www. mhlw.go.jp/stf/seisakunitsuite/bunya/ html,.. http :// or.jp/entry/1356#p09,. Sada KE, Amano K, Uehara R, et al : A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitischurgstrauss in Japan. Mod Rheumatol 2014 ; 24 : ANCA :. Wiik A : Rational use of ANCA in the diagnosis of vasculitis. RheumatologyOxford2002 ; 41 : Sugiyama K, Sada KE, Kurosawa M, et al : Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan. Clinical and Exp Nephrol 2013 ; 17 : 5158.,,, :MPOANCA ANCA. ; :.,, :MPOANCA. ; :.,, :..,, :. ; :. Weidner S, Geuss S, HafeziRachti S, et al : ANCAassociated vasculitis with renal involvement : an outcome analysis. Nephrol, Dial, Transplant 2004 ; 19 : Hauer HA, Bajema IM, van Houwelingen HC, et al : Renal histology in ANCAassociated vasculitis : differences between diagnostic and serologic subgroup. Kidney Int

6 11986 AAV ; 61 : Woodworth TG, Abuelo JG, Austin HA, et al : Severe glomerulonephriris with late emergence of classic Wegener s granulomatosis. Report of 4 cases and review of the literature. Medicine 1987 ; 66 : Koyama A, Yamagata K, Makino H, et al : A nationwide survey of rapidly progressive glomerulonephritis in Japan : etiology, prognosis and treatment diversity. Clin Exp Nephrol 2009 ; 13 : Sada KE, Yamamura M, Harigai M, et al : Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibodyassociated vasculitis in a nationwide, prospective, inception cohort study. Arthritis Res Ther 2014 ; 16 : R101.,, : EGPA. : :. Sada KE, Yamamura M, Harigai M, et al : Issues associated with the Ministry of Health, Labour and Welfare diagnostic criteria for antineutrophil cytoplasmic antibody associated vasculitides : Reclassification of patients in the prospective cohort study of Remission Induction Therapy in Japanese patients with ANCAassociated vasculitides according to the MHLW criteria. Mod Rheumatol 2015 ; 25 : Luqmani RA, Bacon PA, Moots RJ, et al : Birmingham vasculitis activity scorebvasin systemic vasculitis. QJM 1994 ; 87 : Mukhtyar C, Lee R, Brown D, et al : Modification and validation of the Birmingham Vasculitis Activity Score version 3. Ann Rheum Dis 2009 ; 68 : EUVAS : MYCYC clinical trial protocol European Vasculitis Study Group trial Ver : pp 18. Mukhtyar C, Guillevin L, Cid MC, et al : EULAR recommendation for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009 ; 68 : Lapraik C, Watts R, Bacon P, et al : BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. RheumatologyOxford2007 ; 46 : Mukhtyar C, Guillevin L, Cid MC, et al : EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 2009 ; 68 : Lapraik C, Watts R, Bacon P, et al : BSR and BHPR guidelines for the management of adults with ANCA accosiated vasculitis. RheumatologyOxford2007 ; 46 : :. ;. :RiCRAV AAV. ; :. :ANCA, ANCA ANCA. ;.,,, :ANCA ANCA Otitis media with ANCA associated vasculitisomaav. Otology Japan 2013 ; 23 : ,,, : OMAAV. Otology Japan 2015 ; 25 : Yoshida N, Iino Y : Pathogenesis and diagnosis of otitis media with ANCA associated vasculitis. Allergol Int 2014 ; 63 :

2009 7 4 283 1 CHCC ( 1994) 3 Watts (2007) (Wegener granulomatosis WG) 1 X ( > 1 ( ( ) ) ) 2 1 / >1 ) (microscopic polyangiitis 2 MPA) > 3 ( ) 3 ( ) 4

2009 7 4 283 1 CHCC ( 1994) 3 Watts (2007) (Wegener granulomatosis WG) 1 X ( > 1 ( ( ) ) ) 2 1 / >1 ) (microscopic polyangiitis 2 MPA) > 3 ( ) 3 ( ) 4 282 Chinese Journal of Otology Vol. 7 No.4 2009 ANCA (100853) R543 R593.9 A 1672-2922(2009)04-0282-014 (anti-neutrophil cytoplasmic autoantibodies ANCA) (ANCA associated systemic vasculitis AASV) ANCA