Guillevin Loic
Department of Internal Medicine, Referral Centre for Rare Systemic and Autoimmune Diseases, Vasculitis and Scleroderma, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
Clin Exp Rheumatol. 2017 Mar-Apr;35 Suppl 103(1):199-201. Epub 2017 Apr 5.
ANCA-associated vasculitis(AAV) covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined. Herein, we review only maintenance treatments for AAVs.
抗中性粒细胞胞浆抗体相关性血管炎(AAV)涵盖一组系统性坏死性血管炎,其特征为小血管炎症,有时伴有肉芽肿,并与针对中性粒细胞胞浆蛋白酶(蛋白酶3或髓过氧化物酶)的自身抗体相关。若不及时诊断和治疗,AAV可能致命,目前的治疗方式可使大多数患者病情缓解。然而,复发风险仍然很高,需要长期进行免疫抑制或免疫调节维持治疗,而最佳疗程仍未确定。在此,我们仅综述AAV的维持治疗。