Department of Renal Medicine, Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK Department of Clinical Sciences, Rheumatology, Lund University, Lund, Sweden.
Service de Medecine Interne, Hôpital Edouard Herriot, Lyon, France.
Ann Rheum Dis. 2016 Feb;75(2):396-401. doi: 10.1136/annrheumdis-2014-206095. Epub 2014 Dec 2.
Conventional treatment of eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss) with glucocorticoids, with or without additional immunosuppressive drugs, is limited by partial efficacy, frequent toxicity and a high relapse rate. Rituximab is a licensed treatment for granulomatosis with polyangiitis and microscopic polyangiitis and is of potential benefit to patients with EGPA.
Patients with EGPA who received rituximab as single or repeated courses were identified from four vasculitis centres. Standardised data collection was performed, including disease activity status and adverse events, at the time of initial treatment and after 6 and 12 months. Remission was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as a ≥50% reduction in BVAS compared with baseline.
41 patients (21 women) with EGPA treated with rituximab between 2003 and 2013 were identified. 15 (37%) had refractory, 21 (51%) relapsing and 5 (12%) new onset disease. 19 received a single course and 22 received repeat-dose rituximab to prevent relapse. By 6 months, 83% improved with remission in 34% and partial response in 49%, and by 12 months 49% were in remission and 39% had a partial response. Prednisolone doses decreased in all patients by 6 and 12 months. Antineutrophil cytoplasmic antibody positivity at baseline was associated with a higher remission rate at 12 months. Adverse events included 15 infections (6 were severe).
The treatment of EGPA with rituximab resulted in high rates of improvement and reduced requirement of prednisolone. Rituximab may be considered for the treatment of EGPA.
嗜酸性肉芽肿性多血管炎（EGPA）（Churg-Strauss）的常规治疗采用糖皮质激素，联合或不联合其他免疫抑制剂，但疗效有限，毒性频繁且复发率高。利妥昔单抗是获批用于治疗肉芽肿性多血管炎和显微镜下多血管炎的药物，对 EGPA 患者可能有益。
从四个血管炎中心确定了接受利妥昔单抗单药或重复疗程治疗的 EGPA 患者。在初始治疗时以及治疗后 6 个月和 12 个月时，进行标准化数据收集，包括疾病活动状态和不良事件。缓解定义为 Birmingham Vasculitis Activity Score（BVAS）为 0，部分缓解定义为与基线相比 BVAS 降低≥50%。
2003 年至 2013 年间，共确定了 41 例（21 例女性）接受利妥昔单抗治疗的 EGPA 患者。15 例（37%）为难治性，21 例（51%）为复发性，5 例（12%）为新发疾病。19 例患者接受了单疗程治疗，22 例患者接受了重复剂量利妥昔单抗以预防复发。治疗 6 个月时，83%的患者病情改善，其中 34%达到缓解，49%达到部分缓解；治疗 12 个月时，49%的患者达到缓解，39%达到部分缓解。所有患者在 6 个月和 12 个月时的泼尼松剂量均减少。基线时抗中性粒细胞胞质抗体阳性与 12 个月时的缓解率更高相关。不良事件包括 15 例感染（6 例为严重感染）。
利妥昔单抗治疗 EGPA 可获得较高的改善率，并降低泼尼松的需求。利妥昔单抗可考虑用于 EGPA 的治疗。
