ANCA相关性血管炎肾损伤的诊疗进展

摘要/Abstract

摘要： 抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic autoantibody，ANCA)相关性血管炎(ANCA-associated vasculitis，AAV)是一类以小血管坏死性炎症和ANCA存在为特征的自身免疫性疾病，肾脏是常见受累的器官，典型表现为急性肾损伤。AAV的肾脏基本病理变化以寡免疫复合物沉积的坏死性新月体肾炎为特征。近年来越来越多的机制研究表明，补体、尤其是补体旁路途径的活化在AAV的发病中发挥了重要的作用。诱导缓解治疗是临床治疗 AAV的关键。糖皮质激素联合环磷酰胺是标准治疗方案，利妥昔单抗则可作为难治性血管炎的替代治疗。对环磷酰胺耐药、反复发作的AAV患者，吗替麦考酚酯联合糖皮质激素治疗可能有效。激素冲击联合血浆置换治疗重症AAV疗效肯定。以补体C5a受体为靶点的研究，初步结果显示出了其治疗AAV的有效性和安全性。本文结合病理及发病机制，就近年来关于AAV诱导治疗方面的研究进展作简要综述。

Abstract: Anti-neutrophil cytoplasmic autoantibody(ANCA) associated vasculitis(AAV) is a kind of autoimmune diseases characterized by necrotizing inflammation of small vessels and presence of ANCA， and can involve the whole body and multiple systems.The kidney is a commonly involved organ， typically characterized by acute kidney injury， with rapid progress and poor prognosis.The basic pathological changes in the kidney of AAV are characterized by necrotic crescentic nephritis deposited by oligo-immunocomplex. In the recent years， accumulating evidence from both experimental and clinic study has strongly incriminated alternative pathway of complement activation as critically important in the pathogenesis of AAV. Induction remission therapy is the key of clinical treatment for AAV. Glucocorticoids in combination with cyclophosphamide are standard protocols. Rituximab can be recommended as an alternative to the treatment of refractory AAV. Mycophenolate mofetil combined with glucocorticoid therapy may be effective in patients with CTX-resistant and recurrent AAV. High dosage methylprednisolone combined with plasmapheresis treatment have significant effects on patients with severe AAV. Preliminary data of clinical trials have demonstrated effectiveness and safety of C5a receptor blockade in patients with AAV．In this review， combined with pathology and pathogenesis， a brief review of recent research progress on AAV induction remission therapy is presented.

耿毓汕. ANCA相关性血管炎肾损伤的诊疗进展[J]. .

GENG Yushan. Advances on Induction Remission Therapy of ANCA-associated Vasculitis with Renal Injury[J]. .

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