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[抗干扰素-γ自身抗体诱导的成人起病免疫缺陷:13例报告]

[Adult-onset immunodeficiency induced by anti-interferon-γ autoantibodies: report of 13 cases].

作者信息

Nie Y D, Pan S Q, Wang H, Wang T Y, Feng Y Y, Zhang Y, Guo F P, Liu H T, Zhang B Q, Zhao Y, Zhang T, Wang L, Fan J P, Tian X L, Zhang L Q, Wang J L

机构信息

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.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2023 Feb 12;46(2):144-150. doi: 10.3760/cma.j.cn112147-20220725-00625.

Abstract

To investigate the clinical characteristics of a group of patients with adult-onset immunodeficiency (AOID) induced by anti-interferon-γ autoantibodies (AIGA). 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. 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. AOID also affects patients visiting northern China hospitals. AIGA screening is recommended among patients with disseminated NTM infections or recurrent infections.

摘要

探讨一组由抗干扰素-γ自身抗体(AIGA)诱导的成人起病免疫缺陷(AOID)患者的临床特征。纳入了2020年10月至2022年4月在中国北方一家医疗中心(北京协和医院)的13例AOID患者。收集了包括临床表现、实验室检查结果、感染部位和病原体的数据。13例患者中,5例为男性。发病年龄中位数为47(14至71)岁。从症状出现到诊断的中位时间为4年(1至8年)。4例患者来自中国北方,9例来自中国南方。常见症状包括淋巴结病(13/13)、发热(12/13)、呼吸道症状(12/13)和体重减轻(11/13)。实验室检查显示白细胞计数升高(9/13)、中性粒细胞计数及比例升高(9/13)、红细胞沉降率(ESR)升高(12/13)和C反应蛋白(CRP)升高(11/13)。诊断时AIGA的血浆滴度中位数为5681(3194,13246)。感染部位包括肺部(12/13)、淋巴结(9/13)、骨骼和关节(9/13)、皮肤和软组织(7/13)、血流和骨髓(4/13)以及腺体(3/13)。大多数患者有非结核分枝杆菌(NTM)(12/13)感染。7例患者有不止一种病原体。AOID也影响到在中国北方医院就诊的患者。对于播散性NTM感染或反复感染的患者,建议进行AIGA筛查。

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