Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune Diseases, Paris University, Paris, France.
Department of Medicine and Surgery, Università degli Studi di Milano-Bicocca, Milano/Monza, Italy.
Rheumatology (Oxford). 2021 Sep 1;60(9):4355-4360. doi: 10.1093/rheumatology/keaa805.
Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA.
We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status.
ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients.
PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.
仅有三分之一的嗜酸性肉芽肿性多血管炎（EGPA）患者的抗中性粒细胞胞质抗体（ANCA）阳性，主要针对髓过氧化物酶（MPO）。在 EGPA 中很少发现针对蛋白酶 3（PR3）的 ANCA。我们旨在研究 PR3-ANCA 在 EGPA 中的意义。
我们建立了一个回顾性的欧洲多中心队列，包括 845 名患者。根据 ANCA 状态分析和比较了基线特征和结局。
845 名患者中有 734 名患者的 ANCA 状态可查：508 名（69.2%）为 ANCA 阴性，210 名（28.6%）为 MPO-ANCA，16 名（2.2%）为 PR3-ANCA。在基线时，与 MPO-ANCA 和 ANCA 阴性患者相比，PR3-ANCA 患者较少有活动性哮喘（69%比 91%和 93%，P=0.003）和周围神经病（31%比 71%和 47%，P<0.0001），更常见皮肤表现（63%比 38%和 34%，P=0.03）和肺结节（25%比 10%和 8%，P=0.046），且中位嗜酸性粒细胞计数较低（1450 比 5400 和 3224/mm3，P<0.0001）。与 ANCA 阴性患者相比，PR3-ANCA（风险比 6.05，P=0.005）和 MPO-ANCA 患者（风险比 1.88，P=0.0002）的血管炎无复发生存时间更短。
与 MPO-ANCA 和阴性 ANCA 患者相比，PR3-ANCA EGPA 患者存在差异，与肉芽肿性多血管炎具有相似的临床特征。这表明 PR3-ANCA EGPA 可能是 PR3-ANCA 相关血管炎的一种特殊形式。
