表现为重度哮喘的IgG4相关性疾病1例  

IgG4-related disease manifested as severe asthma: a case report

作　　者：李霁[1] 刘湘宁 陈茹萱 邵池[3] 徐凯[4] 黄慧[3] 徐作军[3] Li Ji;Liu Xiangning;Chen Ruxuan;Shao Chi;Xu Kai;Huang Hui;Xu Zuojun(Department of Pulmonary and Critical Care Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Internal Medical Department,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China;Radiological Department,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院内科,北京100730 [3]中国医学科学院北京协和医学院北京协和医院呼吸与危重症医学科,北京100730 [4]中国医学科学院北京协和医学院北京协和医院放射科,北京100730

出　　处：《中华结核和呼吸杂志》2023年第3期278-281,共4页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：中国医学科学院中央级公益性科研院所基本科研业务费专项资金资(2019XK320037);中央高水平医院临床科研业务费(2022-PUMCH-A-009, 2022-PUMCH-C-069)。

摘　　要：IgG4-相关性疾病(IgG4-related disease, IgG4-RD)是一大类以淋巴细胞、浆细胞浸润为主的慢性炎症和病变部位纤维化为特征的系统性疾病, 呼吸系统受累并不少见, 但其临床表现并无特异性且异质性很大, 易与其他呼吸系统疾病混淆。本文报道1例以长达7年的重度哮喘为主要表现患者, 伴有重度过敏性鼻炎、外周血嗜酸性粒细胞增多、血清总IgE及IgG4均明显升高;最终经颌下腺及颈部淋巴结活检确诊IgG4-RD, 经口服激素和免疫抑制剂后控制病情。IgG4-related disease(IgG4-RD)is a systemic inflammatory disease which is characterized by chronic inflammation and fibrosis in the involved tissues/organs,predominantly infiltrated with lymphocytes and plasma cells.The respiratory system is usually involved in IgG4-RD,however,the respiratory clinical manifestations are varied from case to case,and they might mimic other respiratory diseases.We reported a patient who had been diagnosed with severe asthma for around 7 years.He was also suffered from allergic rhinitis.He had peripheral eosinophilia and elevated serum total IgE and IgG4.Finally,he was diagnosed with IgG4-RD after submaxillary gland resection and cervical lymph node biopsy.His condition was improved significantly after oral corticosteroids and immunosuppressants.

关 键 词：系统性疾病 IGG4 重度哮喘 口服激素 血清总IGE 浆细胞浸润 呼吸系统疾病 过敏性鼻炎 

分 类 号：R562.25[医药卫生—呼吸系统]