嗜酸性肉芽肿性多血管炎30例临床分析

Eosinophilic granulomatosis with polyangiitis: 30 cases report

目的分析嗜酸性肉芽肿性多血管炎(EGPA)的临床特征。方法回顾性分析2005年8月至2017年4月中日友好医院诊治的30例EGPA患者的临床资料,包括临床表现、实验室检查、影像学检查、病理学表现等。结果30例患者中男9例,女21例,年龄(44.5±13.9)岁。26例(86.7%)患有支气管哮喘(简称哮喘),病程45.0(20.0,78.7)个月,以哮喘为首发表现者15例(50.0%)。患有鼻炎/鼻窦炎者27例(90.0%),累及皮肤13例(43.3%)、神经系统10例(33.3%)、心脏8例(26.7%)、肾脏3例(10.0%)、消化系统3例(10.0%)。外周血嗜酸性粒细胞>10%者23例(76.7%);抗中性粒细胞抗体阳性者3例(10.0%),以核周型抗中性粒细胞胞质抗体(p-ANCA)为主。胸部影像多表现为一过性肺浸润影,形态主要表现为磨玻璃影者21例(70.0%)。病理表现为血管外嗜酸性粒细胞浸润、血管炎及血管外肉芽肿,后两者较少见。结论EGPA女性多见,临床表现复杂多样,缺乏特异性。

Objective To improve the understanding of Eosinophilic granulomatosis with polyangiitis (EGPA). Methods A total of 30 EGPA patients from the China-Japan Friendship Hospital between August 2005 and April 2017 were included, and a systematic retrospective study about clinical manifestations, laboratory findings, imaging features, pathology and so on was conducted. Results There were 9 males and 21 females. Mean age was (44.5±13.9) years at diagnosis. Among these patients, 26 (86.7%) had asthma, duration was 45.0 (20.0, 78.7) months, and 15 (50.0%) had asthma as their first symptom. Rhinitis/nasosinusitis occurred in 27 (90.0%), skin lesions in 13 (43.3%), neuropathy in 10 (33.3%), heart disease in 8 (26.7%), renal involvement in 3 (10.0%) and gastro-intestinal manifestations in 3 (10.0%) of the patients. Peripheral blood eosinophil>10% accounted for 23/30 (76.7%). There were 3 patients (10.0%) who had positive anti-neutrophil antibody, and the pattern by immunofluorescence was mainly perinuclear. Migratory infiltrates often observed on the chest radiograph, and the main presentation was ground-glass opacity, amounting for 21/30 (70.0%). The pathological manifestations were extravascular eosinophilic tissue infiltration, necrotizing vasculitis, and extravascular granulomas, and the latter two of which were fewer. Conclusion The manifestations of EGPA are individual, complex, with a lack of pathognomonic symptoms, and female predominated.