嗜酸性肉芽肿性多血管炎146例患者临床特征分析

目的:分析和总结嗜酸性肉芽肿性多血管炎(EGPA)的临床特征。方法:收集2000年至2019年北京协和医院确诊的146例EGPA住院患者资料,回顾性分析其临床表现、实验室检查、辅助检查、治疗、并发症及转归。结果:146例患者中男93例,女53例,年龄(41.7±16.1)岁,发病至确诊时间为0.5~450个月,中位时间18(6,60)个月。以肺部121例(82.9%)]、鼻与鼻窦119例(81.5%)]受累最多见;血清抗中性粒细胞胞质抗体(ANCA)阳性者36例(24.7%),以核周型或髓过氧化物酶ANCA为主。ANCA阳性者肾脏(66.7%比20.9%,P<0.001)、神经系统受累比例(80.6%比51.8%,P<0.001)明显高于ANCA阴性者,更易出现发热(66.7%比40.9%,P<0.05)及视神经病变(8.3%比0,P<0.05),疾病活动度更高中位伯明翰血管炎活动度评分25(18,30)比19(14,24),P=0.001],红细胞沉降率40.5(20.5,82.8)mm/1 h比25.0(13.3,50.8)mm/1 h,P=0.006]和超敏C反应蛋白37.1(11.8,72.9)mg/L比13.5(3.4,66.1)mg/L,P=0.036]显著升高;而ANCA阴性者胸腔积液比例高于ANCA阳性者(20.9%比5.6%,P=0.04)。治疗以糖皮质激素和环磷酰胺为主。12例(8.2%)患者病情缓解,6例(4.1%)患者死亡或放弃治疗。并发症以肺部感染最多见。结论:EGPA是一种少见的血管炎,临床表现与转归异质性强,病死率高。

Clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis

Objective To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis(EGPA).Methods The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively,including clinical manifestations,laboratory results,treatment,complications and outcome at discharge.Birmingham Vasculitis activity score-V3(BVAS-V3)was used to evaluate disease activity.Results The ratio of male to female was 1.8∶1 with average age(41.7±16.1)year-old.The median time from disease onset to diagnosis was 18(6,60)months(0.5~450).The most common clinical manifestations were lung121(82.9%)]and nose/paranasal sinuses119(81.5%)]involvement.The positive rate of anti-neutrophil cytoplasmic antibody(ANCA)was 24.7%,mainly peripheral(P)-ANCA/myeloperoxidase(MPO)-ANCA.Compared with ANCA-negative patients,the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement(66.7%vs.20.9%,80.6%vs.51.8%,P<0.001),fever and optic neuropathy(66.7%vs.40.9%,8.3%vs.0,P<0.05),more active diseasemedian BVAS-V325(18,30)vs.19(14,24),P=0.001]and more elevated erythrocyte sedimentation rate40.5(20.5,82.8)mm/1h vs.25.0(13.3,50.8)mm/1h,P=0.006]and C-reactive protein37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L,P=0.036].More ANCA-negative patients had pleural effusion(20.9%vs.5.6%,P<0.04)compared with ANCA-negative patients.Pulmonary infection was the most common complication.A total of 12 EGPA patients(8.2%)achieved remission and 6 patients(4.1%)died or discharged themselves from the hospital.Conclusion EGPA is a rare small vessel vasculitis.The clinical manifestations and outcomes are heterogenous.The mortality rate of EGPA is high.