抗干扰素γ自身抗体介导的成人起病免疫缺陷症13例分析  

Adult-onset immunodeficiency induced by anti-interferon-γautoantibodies:report of 13 cases

作　　者：聂永都 潘思琪 王瀚 王亭谕 冯芸颖 张颖 郭伏平 刘慧婷 张冰清 赵颖 张婷 王珞 范俊平 田欣伦 张林琦[3] 王京岚 Nie Yongdu;Pan Siqi;Wang Han;Wang Tingyu;Feng Yunying;Zhang Ying;Guo Fuping;Liu Huiting;Zhang Bingqing;Zhao Ying;Zhang Ting;Wang Luo;Fan Junping;Tian Xinlun;Zhang Linqi;Wang Jinglan(Department of Pulmonary and Critical Care Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;Graduate School,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;School of Medicine,Tsinghua University,Beijing 100084,China;Department of Medical Records,Peking Union Medical College Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;Department of International Medical Service,Peking Union Medical College Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;Department of Infectious Diseases,Peking Union Medical College Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;Department of General Internal Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Science&Peking Union Medical College,Beijing 100730,China;Department of Clinical Laboratory,Peking Union Medical College Hospital,Chinese Academy of Medical Science&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、北京协和医院呼吸与危重症医学科,北京100730 [2]中国医学科学院、北京协和医学院研究生院,北京100730 [3]清华大学医学院,北京100084 [4]中国医学科学院、北京协和医学院、北京协和医院病案科,北京100730 [5]中国医学科学院、北京协和医学院、北京协和医院国际医疗部,北京100730 [6]中国医学科学院、北京协和医学院、北京协和医院感染内科,北京100730 [7]中国医学科学院、北京协和医学院、北京协和医院普通内科,北京100730 [8]中国医学科学院、北京协和医学院、北京协和医院检验科,北京100730

出　　处：《中华结核和呼吸杂志》2023年第2期144-150,共7页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：中国医学科学院医学与健康科技创新工程项目(2021-I2M-1-048)。

摘　　要：目的描述和分析一组抗干扰素γ自身抗体(AIGA)介导的成人起病免疫缺陷症(AOID)患者的临床特征。方法收集2020年10月1日至2022年4月30日,在北京协和医院确诊的AOID患者共13例,对其临床表现、实验室检查、感染部位及病原种类进行描述和分析。结果13例患者中男性5例,起病中位年龄47岁(14~71岁),首发症状到确诊中位时间4年(1~8年);籍贯北方地区4例,南方地区9例。常见临床表现为淋巴结肿大(13/13)、发热(12/13)、咳嗽等呼吸道症状(12/13)、体重下降(11/13)。常见实验室检查异常为白细胞计数升高(9/13),中性粒细胞计数及占比升高(7/13),红细胞沉降率升高(12/13)以及C反应蛋白升高(11/13)。诊断时血浆AIGA滴度中位数为5681(3194,13246)。常见感染受累部位为肺(12/13)、淋巴结(9/13)、骨或关节(9/13)、皮肤软组织(7/13)、血流及骨髓(4/13)、腺体(3/13)。最常见的病原为非结核分枝杆菌(12/13),2种及以上病原感染多见(9/13)。结论我国AIGA介导的AOID患者同样见于北方医疗机构就医人群,在播散性非结核分枝杆菌感染或反复感染等患者中应重视AIGA的筛查。Objective To investigate the clinical characteristics of a group of patients with adult-onset immunodeficiency(AOID)induced by anti-interferon-γautoantibodies(AIGA).Methods Thirteen cases of AOID in a northern China medical center(Peking Union Medical College Hospital)from October 2020 to April 2022 were included.Data comprising clinical manifestations,laboratory results,infection sites and pathogens were collected.Results Among the 13 patients,5 were male.The median age of disease onset was 47(14 to 71)years.The median time from symptom onset to diagnosis was 4 years(1 to 8 years).Four patients were from northern China,and 9 from southern China.Common symptoms included lymphadenopathy(13/13),fever(12/13),respiratory tract symptoms(12/13),and weight loss(11/13).Laboratory tests showed increased levels of white blood cell count(9/13),neutrophil count and proportion(9/13),erythrocyte sedimentation rate(ESR)(12/13),and C reactive protein(CRP)(11/13).The median plasma titers of AIGA upon diagnosis were 5681(3194,13246).Sites of infection included lungs(12/13),lymph nodes(9/13),bones and joints(9/13),skin and soft tissue(7/13),blood flow and bone marrow(4/13),and glands(3/13).Most patients had nontuberculous mycobacteria(NTM)(12/13)infection.Seven patients had more than one pathogen.Conclusions AOID also affects patients visiting northern China hospitals.AIGA screening is recommended among patients with disseminated NTM infections or recurrent infections.

关 键 词：干扰素Γ 自身抗体 免疫缺陷 非结核分枝杆菌 

分 类 号：R593.3[医药卫生—内科学]