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会计学1第1页/共30页Jennette et al.Arthritis Rheum 1994;37:187-192ANCA相关性血管炎（AAV）1994年Chapel Hill 会议命名第2页/共30页2011 15th International vasculitis&ANCA worksho European League Against Rheumatism(EULAR)European Vasculitis Study Group(EUVAS)第3页/共30页ANCA-associated Vasculitis（AAV）2012 Chapel Hill Consensus Conference Vasculitis Nomenclature第4页/共30页原发性系统性小血管炎原发性系统性小血管炎第5页/共30页第6页/共30页老年人可以肺间质纤维化首发，且全身小血管炎无明显活动老年人可以肺间质纤维化首发，且全身小血管炎无明显活动MPA主要为肺部浸润影、肺间质纤维化、弥漫性肺泡出血WG肺结节性病变第7页/共30页第8页/共30页第9页/共30页第10页/共30页第11页/共30页第12页/共30页第13页/共30页P-ANCA 核周型（核周型（MPA）MPO(髓过氧化物酶）髓过氧化物酶）ANCAANCA滴度与病情活动相关滴度与病情活动相关 C-ANCA 胞浆型胞浆型（WG）PR3（丝氨酸蛋白酶（丝氨酸蛋白酶3）第14页/共30页第15页/共30页一元论一元论多系统性损害，尤其肺、肾损害详细的病史及查体血清学检查：ANCA、ESR、CRP、自身抗体、RF、补体、蛋白电泳治疗确诊血管炎 组织活检心、肺、肾、神经系统检查，明确系统损害的范围和程度 金标准第16页/共30页第17页/共30页BVAS达到达到25即为高危即为高危第18页/共30页诱导缓解治疗长期保护肾功能减少复发维持治疗尽快控制炎症争取完全缓解治疗目标提高生存率、保存靶器官功能、减少副作用复发治疗尽快控制炎症争取完全缓解第19页/共30页13.1.1:We recommend that cyclophosphamide and corticosteroids be used as initial treatment.(1A)13.1.2:We recommend that rituximab and corticosteroids be used as an alternative initial treatment in patients without severe disease or in whom cyclophosphamide is contraindicated.(1B)KDIGO-AAVKDIGO-AAV治疗指南-1-1第20页/共30页13.3.1:We recommend maintenance therapy in patients who have achieved remission.(1B)13.3.2:We suggest continuing maintenance therapy for at least 18 months in patients who remain in complete remission.(2D)13.3.3:We recommend no maintenance therapy in patients who are dialysis-dependent and have no extrarenal manifestations of disease.(1C)KDIGO-AAVKDIGO-AAV治疗指南-2-2第21页/共30页13.4.1:We recommend azathioprine 12 mg/kg/d azathioprine 12 mg/kg/d orally as maintenance therapy.(1B)13.4.2:We suggest that MMF,up to 1 g twice daily,be used for maintenance therapy in patients who are allergic to,or intolerant of,azathioprine.(2C)13.4.3:We suggest trimethoprim-sulfamethoxazol trimethoprim-sulfamethoxazole as an adjunct to maintenance therapy in patients with upper respiratory tract disease.(2B)13.4.4:We suggest methotrexate(initially 0.3 mg/kg/wk,methotrexate(initially 0.3 mg/kg/wk,maximum 25 mg/wk)maximum 25 mg/wk)for maintenance therapy in patients intolerant of azathioprine and MMF,but not if GFR is 60 ml/min per 1.73m2.(1C)13.4.5:We recommend not using etanercept not using etanercept as adjunctive therapy.(1A)KDIGO-AAVKDIGO-AAV治疗指南-3-3第22页/共30页13.5.1:We recommend treating patients with severe relapse of ANCA vasculitis(life-or organ-threatening)according to the same guidelines as for the initial therapy(see Section 13.1).(1C)13.5.2:We suggest treating other relapses of ANCA vasculitis by reinstituting immunosuppressive therapy or increasing its intensity with agents other than cyclophosphamide,including instituting or increasing dose of corticosteroids,with or without azathioprine or MMF.(2C)13.6.1:In ANCA GN resistant to induction therapy with cyclophosphamide and corticosteroids,we recommend the addition of rituximab(1C),and suggest i.v.immunoglobulin(2C)or plasmapheresis(2D)as alternatives.KDIGO-AAVKDIGO-AAV治疗指南-4-4第23页/共30页13.7.1:We suggest not changing immunosuppression based on changes in ANCA titer alone.(2D)13.8.1:We recommend delaying transplantation until patients are in complete extrarenal remission for 12 months.(1C)13.8.2:We recommend not delaying transplantation for patients who are in complete remission but are still ANCA-positive.(1C)KDIGO-AAVKDIGO-AAV治疗指南-5-5第24页/共30页EUVASEUVAS治疗建议第25页/共30页第26页/共30页预预 后后第27页/共30页第28页/共30页第29页/共30页
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