Comarmond Cloé, Pagnoux Christian, Khellaf Mehdi, Cordier Jean-François, Hamidou Mohamed, Viallard Jean-François, Maurier François, Jouneau Stéphane, Bienvenu Boris, Puéchal Xavier, Aumaître Olivier, Le Guenno Guillaume, Le Quellec Alain, Cevallos Ramiro, Fain Olivier, Godeau Bertrand, Seror Raphaèle, Dunogué Bertrand, Mahr Alfred, Guilpain Philippe, Cohen Pascal, Aouba Achille, Mouthon Luc, Guillevin Loïc
Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris 5, Paris, France.
Arthritis Rheum. 2013 Jan;65(1):270-81. doi: 10.1002/art.37721.
Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), with limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken to describe the main characteristics of a larger patient cohort and their long-term outcomes.
A retrospective study of EGPA patients in the French Vasculitis Study Group cohort who satisfied the American College of Rheumatology criteria and/or Chapel Hill definitions was conducted. Patient characteristics and outcomes were compared according to ANCA status and year of diagnosis.
We identified 383 patients diagnosed between 1957 and June 2009 (128 [33.4%] before 1997 or earlier) and followed up for a mean±SD of 66.8±62.5 months. At diagnosis, their mean±SD age was 50.3±15.7 years, and 91.1% had asthma (duration 9.3±10.8 years). Main manifestations included peripheral neuropathy (51.4%); ear, nose, and throat (ENT) signs (48.0%); skin lesions (39.7%); lung infiltrates (38.6%); and cardiomyopathy (16.4%). Among the 348 patients tested at diagnosis for ANCA, the 108 ANCA-positive patients (31.0%) had significantly more frequent ENT manifestations, peripheral neuropathy, and/or renal involvement, but less frequent cardiac manifestations, than the ANCA-negative patients. Vasculitis relapses occurred in 35.2% of the ANCA-positive versus 22.5% of the ANCA-negative patients (P=0.01), and 5.6% versus 12.5%, respectively, died (P<0.05). The 5-year relapse-free survival rate was 58.1% (95% confidence interval [95% CI] 45.6-68.6) for ANCA-positive and 67.8% (95% CI 59.8-74.5) for ANCA-negative patients (P=0.35). Multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse.
The characteristics and long-term outcomes of EGPA patients differ according to their ANCA status. Although EGPA relapses remain frequent, mortality has declined, at least since 1996.
早期关于嗜酸性肉芽肿性多血管炎（Churg-Strauss）（EGPA）的研究，因患者数量和随访时间有限，表明诊断时的临床表现因抗中性粒细胞胞浆抗体（ANCA）状态而异，但结局并无差异。本研究旨在描述一个更大患者队列的主要特征及其长期结局。
对法国血管炎研究组队列中符合美国风湿病学会标准和/或 Chapel Hill 定义的 EGPA 患者进行回顾性研究。根据 ANCA 状态和诊断年份比较患者特征和结局。
我们确定了 1957 年至 2009 年 6 月期间诊断的 383 例患者（1997 年或更早之前诊断的有 128 例[33.4%]），平均随访时间为 66.8±62.5 个月。诊断时，他们的平均年龄为 50.3±15.7 岁，91.1%有哮喘（病程 9.3±10.8 年）。主要表现包括周围神经病变（51.4%）；耳、鼻、喉（ENT）体征（48.0%）；皮肤病变（39.7%）；肺部浸润（38.6%）；以及心肌病（16.4%）。在诊断时检测 ANCA 的 348 例患者中，108 例 ANCA 阳性患者（31.0%）的 ENT 表现、周围神经病变和/或肾脏受累更为频繁，但心脏表现较少，低于 ANCA 阴性患者。ANCA 阳性患者中有 35.2%发生血管炎复发，而 ANCA 阴性患者为 22.5%（P=0.01），死亡率分别为 5.6%和 12.5%（P<0.05）。ANCA 阳性患者的 5 年无复发生存率为 58.1%（95%置信区间[95%CI]45.6 - 68.6），ANCA 阴性患者为 67.8%（95%CI 59.8 - 74.5）（P=0.35）。多变量分析确定心肌病、年龄较大以及 1996 年或之前诊断为死亡的独立危险因素，诊断时嗜酸性粒细胞计数较低可预测复发。
EGPA 患者的特征和长期结局因其 ANCA 状态而异。尽管 EGPA 复发仍然频繁，但至少自 1996 年以来死亡率有所下降。
