Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon.
Claude Bernard University Lyon 1, Lyon, France.
Curr Opin Pulm Med. 2020 Sep;26(5):496-506. doi: 10.1097/MCP.0000000000000693.
To summarize the latest publications and provide a practical overview of treatment strategies for lung vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs).
In patients with severe ANCA-associated vasculitis, plasma exchange, as adjunctive therapy to standard treatment, is not associated with improved survival or reduced risk of end-stage kidney disease. A regimen with reduced dose of glucocorticoids is equally effective to induce remission as a standard regimen. In patients without organ or life-threatening disease, mycophenolate mofetil can be used in combination with oral glucocorticoid therapy to induce remission, however, with a higher risk of relapse than when using rituximab or cyclophosphamide. For maintenance of remission, a tailored regimen of rituximab infusion was equivalent to a fixed regimen, with fewer perfusions. Belimumab, a human IgG1(Equation is included in full-text article.)monoclonal antibody against B-lymphocyte stimulator, did not decrease the relapse rate when added to azathioprine and glucocorticoids. Avacopan, a complement C5a receptor inhibitor, was effective in replacing high-dose glucocorticoids in achieving complete remission of vasculitis.
Significant advances have been made in the treatment strategy to both induce remission and maintain remission in patients with ANCA-associated vasculitis. The choice should take into consideration efficacy, cost-effectiveness, safety profile, ease of use, and possibility of individual tailoring of treatment.
总结抗中性粒细胞胞浆抗体(ANCA)相关性血管炎的最新文献,并提供治疗策略的实用概述。
在重症 ANCA 相关性血管炎患者中,血浆置换作为标准治疗的辅助疗法,与改善生存或降低终末期肾病风险无关。与标准方案相比,使用减少剂量的糖皮质激素的方案同样能有效诱导缓解。对于无器官或危及生命疾病的患者,霉酚酸酯可与口服糖皮质激素联合用于诱导缓解,但与使用利妥昔单抗或环磷酰胺相比,复发风险更高。为了维持缓解,利妥昔单抗输注的个体化方案与固定方案等效,输注次数更少。贝鲁单抗是一种针对 B 淋巴细胞刺激物的人 IgG1 单克隆抗体,与硫唑嘌呤和糖皮质激素联合使用并未降低复发率。补体 C5a 受体抑制剂阿伐考帕在替代大剂量糖皮质激素以实现血管炎完全缓解方面是有效的。
在诱导和维持 ANCA 相关性血管炎患者缓解方面,治疗策略取得了重大进展。选择时应考虑疗效、成本效益、安全性、易用性以及个体化治疗的可能性。