Guillevin Loïc
Hôpital Cochin, 27, rue du Faubourg Saint-Jacques,75679 Paris Cedex 14, France.
Bull Acad Natl Med. 2008 Jun-Jul;192(6):1175-87; discussion 1188.
Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) mainly affect small vessels. These disorders comprise crescentic glomerulonephritis, Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Treatment of ANCA-associated vasculitides should be adapted to the type and severity of clinical manifestations, and to certain patient characteristics. Corticosteroids and immunosuppressants are compulsory in Wegener's granulomatosis, but steroids alone can be prescribed to patients with Churg-Strauss syndrome and microscopic polyangiitis without factors of poor prognosis. New biotherapies are showing promise in selected patients but need to be evaluated in the long term. Immunosuppressant and steroid dose reductions, and widespread use of pulse cyclophosphamide instead of oral treatment, have improved patient outcome. Remission is currently obtained in more than 90% of cases but relapses are frequent: for example, more than 50% of Wegener's patients relapse three years after remission. New strategies are needed to prevent relapses and to limit adverse drug effects.
与抗中性粒细胞胞浆抗体（ANCA）相关的血管炎主要累及小血管。这些疾病包括新月形肾小球肾炎、韦格纳肉芽肿病、显微镜下多血管炎和变应性肉芽肿性血管炎（Churg-Strauss综合征）。ANCA相关血管炎的治疗应根据临床表现的类型和严重程度以及某些患者特征进行调整。在韦格纳肉芽肿病中，皮质类固醇和免疫抑制剂是必需的，但对于无预后不良因素的变应性肉芽肿性血管炎和显微镜下多血管炎患者，可单独使用类固醇。新的生物疗法在部分患者中显示出前景，但需要长期评估。免疫抑制剂和类固醇剂量的减少以及脉冲环磷酰胺而非口服治疗的广泛应用改善了患者的预后。目前超过90%的病例可实现缓解，但复发频繁：例如，超过50%的韦格纳肉芽肿病患者在缓解三年后复发。需要新的策略来预防复发并限制药物不良反应。
