系统性小血管炎诊治与进展(2016年-4-26).pptVIP

2016-4-26;系统性血管炎是指以血管壁坏死性炎症和/或纤维素样坏死为主要病理特征的一类自身免疫性疾病 病变血管局限于微小动脉、静脉和毛细血管时为小血管炎;原发性系统性小血管炎;2011 15th International vasculitis ANCA worksho European League Against Rheumatism?(EULAR) European?Vasculitis Study Group (EUVAS)…;Microscopic polyangiitis (MPA) Granulomatosis with polyangiitis (GPA) Eosinophilic Granulomatosis with Polyangiitis（CSS）;ANCA阴性不能排除血管炎诊断;临床表现-小血管炎肾损害;临床表现-肺受累的表现;临床表现-头颈部受累;临床??现-其他脏器受累;MPA 临床表现;GPA 临床表现（WG）;eGPA 临床表现(CSS);实验室检查;ANCA;临床表现（症状体征） 不明原因的发热、难以解释的全身症状 多系统损害 进展迅速的脏器功能衰竭（肺肾综合征） 肾脏损害（特别是活动性肾小球肾炎） 肺部病变（浸润、出血、呼衰） 肌肉和关节疼痛 皮肤紫癜及结节性坏死性皮疹 突发神经系统病变，尤其是多发性单神经炎 实验室检查 ESR、CRP、与肾功能下降不平行的贫血 ANCA 病理学证据：金标准;诊断流程;如何判断病情活动？;BVAS（伯明翰血管炎评分系统）;ANCA相关小血管炎的治疗策略;13.1: Initial treatment of pauci-immune focal and segmental necrotizing GN 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);13.3: Maintenance therapy 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);13.4: Choice of agent for maintenance therapy 13.4.1: We recommend azathioprine 1–2 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-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, maximum 25 mg/wk) for maintenance therapy in patients intolerant of azathioprine and MMF, but not if