33例嗜酸性肉芽肿性多血管炎患者的临床特征分析  

Clinical characteristics of 33 patients with eosinophilic granulomatosis with polyangiitis

作　　者：詹明烺 姜林娣 刘云 ZHAN Ming-lang;JIANG Lin-di;LIU Yun(Department of Rheumatology,Zhongshan Hospital(Xiamen Branch),Fudan University,Xiamen 361015,Fujian Province,China;Department of Rheumatology,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院厦门医院风湿免疫科,厦门361015 [2]复旦大学附属中山医院风湿免疫科,上海200032

出　　处：《复旦学报(医学版)》2025年第1期77-82,共6页Fudan University Journal of Medical Sciences

基　　金：国家自然科学基金(82271834)。

摘　　要：目的描述并总结嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)的临床特征,提高对该病的认识。方法收集2017年1月至2022年8月在复旦大学附属中山医院诊治的33例EGPA患者,诊断参照1990年美国风湿病学会(American College of Rheumatology,ACR)关于EGPA的分类标准,回顾性分析患者的临床表现、实验室检查和治疗转归等。结果33例EGPA患者中男性22例(66.7%),女性11例(33.3%),确诊时平均年龄(47.42±15.83)岁。首诊科室以风湿免疫科(23例,69.7%)为主,其次是呼吸内科(6例,18.2%)。临床表现以皮肤受累最多见(皮疹、溃疡、坏疽和梗死,23例,69.7%),其次为哮喘(17例,51.5%)、浸润性肺炎(14例,42.4%)、周围神经病变(9例,27.3%)、血栓形成(9例,27.3%)。所有患者的嗜酸性粒细胞绝对值平均数为(3.43±3.52)×10^(9)/L,有8例(24.2%)患者抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)阳性。和ANCA阴性患者相比,ANCA阳性患者伯明翰血管炎活动度评分(Birmingham Vasculitis Activity Score,BVAS)和嗜酸性粒细胞计数显著升高,肾脏受累更常见(P<0.05)。32例(97%)患者接受糖皮质激素治疗,11例(33.3%)患者使用过生物制剂或托法替尼治疗,6例接受托法替尼治疗的患者中有5例获得了疾病缓解。结论EGPA临床表现多样,ANCA阳性患者疾病活动性更高,应建立多学科的EGPA诊疗体系。Objective To provide a comprehensive description and summary of the clinical characteristics of eosinophilic granulomatosis with polyangiitis(EGPA)in order to enhance understanding of this disease.Methods A total of 33 EGPA patients treated in Zhongshan Hospital,Fudan University,between Jan 2017 and Aug 2022 were included in this retrospective analysis.The diagnosis was based on the 1990 American College of Rheumatology(ACR)classification criteria for EGPA.Clinical manifestations,laboratory examinations,and treatment outcomes of the patients were analyzed.Results Among the 33 EGPA patients,there were 22 males(66.7%)and 11 females(33.3%),with an average age of diagnosis being(47.42±15.83)years old.The most common initial department visited by patients was the rheumatology department(23 cases,69.7%),followed by the respiratory medicine department(6 cases,18.2%).Skin involvement manifested as rash,ulcers,necrosis or gangrene was observed in most cases(23 cases,69.7%),followed by asthma(17 cases,51.5%),infiltrative pneumonia(14 cases,42.4%),peripheral neuropathy(9 cases,27.3%),thrombosis formation(9 cases,27.3%).The mean absolute value of eosinophils in all patients was measured as(3.43±3.52)×10^(9)/L,with eight patients(24.2%)testing positive for antineutrophil cytoplasmic antibody(ANCA).Compared with ANCAnegative patients,ANCA-positive individuals exhibited significantly higher Birmingham Vasculitis Activity Score(BVAS)and eosinophil count,as well as a higher incidence rate of renal involvement(P<0.05).Glucocorticoid therapy was administered in thirty-two patients(97%),while biologics or tofacitinib were given to eleven patients(33.3%),among them six received tofacitinib treatment,of which five achieved disease remission.Conclusion EGPA exhibits a wide range of clinical manifestations,and ANCApositive patients tend to exhibit higher disease activity levels.A multidisciplinary diagnosis and treatment system for EGPA should be established.

关 键 词：嗜酸性肉芽肿性多血管炎(EGPA) 抗中性粒细胞胞浆抗体(ANCA) 托法替尼 

分 类 号：R593.2[医药卫生—内科学]