Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital.
Harvard Medical School.
Curr Opin Rheumatol. 2021 Jan;33(1):8-14. doi: 10.1097/BOR.0000000000000756.
Vasculitides can affect small, medium and/or large vessels, leading to end-organ damage, decreased quality of life and death. Glucocorticoids remain the backbone of treatment for systemic vasculitis but are associated with numerous toxicities. In recent years, the efficacy of glucocorticoid-sparing biologic and novel small molecule therapies has been demonstrated.
In giant cell arteritis, tocilizumab was superior to glucocorticoid monotherapy in maintenance remission and cumulative glucocorticoid exposure and is now approved for the treatment of giant cell arteritis. In addition to the previously demonstrated efficacy of rituximab for remission induction in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, recent trials have also demonstrated its superiority for remission maintenance compared to alternative approaches. Mepolizumab is superior to standard of care alone with regard to remission rates and glucocorticoid-sparing effect in refractory eosinophilic granulomatosis with polyangiitis. Avacopan has shown significant promise in ANCA-associated vasculitis as part of a glucocorticoid-free induction regimen in a recently completed phase 3 trial. Use of biologics in rarer vasculitides remains guided by reports from small case series.
Biologics and other novel therapies have an increasingly important role in the management of systemic vasculitis. Additional studies are needed to define their optimal use and to guide their use in more rare forms of vasculitis.
血管炎可影响小、中、大血管，导致靶器官损伤、生活质量下降和死亡。糖皮质激素仍然是系统性血管炎治疗的基础，但也与许多毒性作用相关。近年来，糖皮质激素节约型生物制剂和新型小分子治疗的疗效已得到证实。
在巨细胞动脉炎中，托珠单抗在维持缓解和累积糖皮质激素暴露方面优于糖皮质激素单药治疗，现已批准用于治疗巨细胞动脉炎。除了先前证明利妥昔单抗在抗中性粒细胞胞质抗体（ANCA）相关性血管炎诱导缓解方面的疗效外，最近的试验还表明，与替代方法相比，其在缓解维持方面具有优越性。美泊利珠单抗在难治性嗜酸性肉芽肿性多血管炎中与标准治疗相比，在缓解率和糖皮质激素节约方面具有优越性。阿伐考帕尼在 ANCA 相关性血管炎中显示出很大的前景，作为最近完成的 3 期试验中无糖皮质激素诱导方案的一部分。在罕见血管炎中使用生物制剂仍然需要根据小病例系列报告来指导。
生物制剂和其他新型疗法在系统性血管炎的治疗中具有越来越重要的作用。需要进一步研究以确定其最佳使用方法，并指导其在更罕见的血管炎形式中的使用。
