嗜酸性肉芽肿性多血管炎37例临床分析

Analysis of clinical characteristics on eosinophilic granulomatosis with polyangiitis:a report of 37 cases

目的:总结嗜酸性肉芽肿性血管炎(EGPA)患者的首发症状、各脏器受累情况、实验室检查结果等数据,以分析其临床特征。方法:回顾性分析2016年1月—2020年1月中日友好医院收治的37例确诊EGPA患者的临床资料。结果:37例患者中,男13例,女24例。发病年龄27~80岁,平均47.64岁。25例(67.6%)患者以哮喘为首发症状。病程中最常见临床表现是哮喘(33例,89.2%)、鼻窦炎(33例,89.2%)、过敏性鼻炎(20例,54.1%)。其中皮肤受累9例(24.3%),外周神经受累7例(18.9%),中枢神经受累2例(5.4%),心脏受累6例(16.2%),消化系统受累3例(8.1%),血管受累5例(13.5%)。肺部CT最常见表现为磨玻璃影及斑片影(33例,89.2%)和支气管壁增厚/支气管扩张(17例,45.9%)。病理表现:36例(97.3%)患者行组织活检,16例(43.2%)病理支持EGPA诊断。治疗上35例(94.6%)患者口服激素治疗,3例(8.1%)联合免疫抑制剂治疗。随访35例患者,中位随访时间为10个月,其中1例死亡、2例在激素减量过程中复发。结论:EGPA临床表现复杂多样,以呼吸系统受累最多见,常以哮喘为首要症状,可累及全身多个器官系统。

Objective:To analyze the clinical characteristics of eosinophilic granulomatous vasculitis(EGPA)by summarizing the data of initial symptoms,organ involvement and laboratory examination.Methods:Clinical data of 37 patients with EGPA admitted to China-Japan Friendship Hospital from January 2016 to January2020 were retrospectively analyzed.Results:Among the 37 patients,13 were males and 24 were females.Their ages of onset was 27~80 years,with an average age of 47.64 years.Asthma was the first symptom in 25 patients(67.6%).The most common clinical manifestations were asthma in 33 cases(89.2%),allergic rhinitis in20 cases(54.1%)and sinusitis in 33 cases(89.2%).The involvement conditions of other organs were as follows:skin in 9 cases(24.3%),peripheral neuropathy in 7 cases(18.9%),central nervous system in 2 cases(5.4%),cardiac involvement in 6 cases(16.2%),digestive system in 3 cases(8.1%),and vascular involvement in 5 cases(13.5%).Laboratory tests showed that 13.5%(5 cases)were positive for ANCA,most of which were p-ANCA. The most common pulmonary CT findings were ground glass shadow and patchy shadow(89.2%)and bronchial wall thickening/bronchiectasis in 17 cases(45.9%).Pathological findings:36 patients underwent biopsy,and 16 patients(43.2%)were pathologically supported by EGPA diagnosis.In terms of treatment,35 patients received oral hormone therapy,3 patients received combined immunosuppressive therapy.Thirty-five patients were followed up for a median time of 10 months,and 1 patient died.Among the remaining34 patients,2 patients relapsed during hormone reduction.Conclusion:The clinical manifestations of EGPA are complex and varied.The respiratory system is most frequently involved,and asthma is often the primary symptom.In addition to eosinophilia,sinusitis and chest imaging manifestations,EGPA may involve multiple organ systems throughout the body.