Clinical immunological characteristics of anti-interferon-γ autoantibodies syndrome: a 3 year prospective cohort study

Siqiao Liang; Hanlin Liang; Xuemei Huang; Xiaona Liang; Ni Chen; Rong Xiao; Zengtao Luo; Quanfang Chen; Xinxin Zhong; Jingmin Deng; Jie Huang; Meihua Li; Meiling Yang; Wen Zeng; Haijuan Tang; Jing Jiang; Shouming Qin; Zhen Wei; Siyao Wu; Yan Ning; Ke Wang; Fu Cao; Jiujin Zhang; Qing Wei; Chengqiong Xu; Honglin Luo; Jian Song; Pei Li; Xiaokai Feng; Chenlu Yang; Jieping Lei; Hongwei Wang; Bin Cao; Zhiyi He

doi:10.1080/22221751.2024.2396887

Emerging Microbes and Infections (Dec 2024)

Clinical immunological characteristics of anti-interferon-γ autoantibodies syndrome: a 3 year prospective cohort study

Siqiao Liang,
Hanlin Liang,
Xuemei Huang,
Xiaona Liang,
Ni Chen,
Rong Xiao,
Zengtao Luo,
Quanfang Chen,
Xinxin Zhong,
Jingmin Deng,
Jie Huang,
Meihua Li,
Meiling Yang,
Wen Zeng,
Haijuan Tang,
Jing Jiang,
Shouming Qin,
Zhen Wei,
Siyao Wu,
Yan Ning,
Ke Wang,
Fu Cao,
Jiujin Zhang,
Qing Wei,
Chengqiong Xu,
Honglin Luo,
Jian Song,
Pei Li,
Xiaokai Feng,
Chenlu Yang,
Jieping Lei,
Hongwei Wang,
Bin Cao,
Zhiyi He

Affiliations

Siqiao Liang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Hanlin Liang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Xuemei Huang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Xiaona Liang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Ni Chen: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Rong Xiao: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Zengtao Luo: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Quanfang Chen: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Xinxin Zhong: Department of Respiratory and Critical Care Medicine, Red Cross Hospital of Yulin City, Yulin, People’s Republic of China
Jingmin Deng: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Jie Huang: Department of Tuberculosis, Nanning Fourth People's Hospital, Nanning, People’s Republic of China
Meihua Li: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Meiling Yang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Wen Zeng: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Haijuan Tang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Jing Jiang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Shouming Qin: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Zhen Wei: Department of Respiratory and Critical Care Medicine, The People Hospital of Hechi, Hechi, People’s Republic of China
Siyao Wu: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Yan Ning: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Ke Wang: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China
Fu Cao: Department of Respiratory and Critical Care Medicine, Red Cross Hospital of Yulin City, Yulin, People’s Republic of China
Jiujin Zhang: Department of Respiratory and Critical Care Medicine, Red Cross Hospital of Yulin City, Yulin, People’s Republic of China
Qing Wei: Department of Respiratory and Critical Care Medicine, The People Hospital of Hechi, Hechi, People’s Republic of China
Chengqiong Xu: Department of Respiratory and Critical Care Medicine, The Second People’s Hospital of Qinzhou, Qinzhou, People’s Republic of China
Honglin Luo: Institute of Oncology, Guangxi Academy of Medical Sciences, Nanning, People’s Republic of China
Jian Song: Institute of Cardiovascular Sciences, Guangxi Academy of Medical Sciences, The People’s Hospital of Guangxi Zhuang Autonomous Region, Nanning, People’s Republic of China
Pei Li: Infectious Diseases Division, KingMed Diagnostics, Guangzhou, People’s Republic of China
Xiaokai Feng: Department of Pulmonary and Critical Care Medicine, Beijing Chao-yang Hospital, Beijing, People’s Republic of China
Chenlu Yang: Department of Epidemiology and Biostatistics, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine, Peking Union Medical College, Beijing, People’s Republic of China
Jieping Lei: Department of Clinical Research and Data Management, Center of Respiratory Medicine, China–Japan Friendship Hospital, Beijing, People’s Republic of China
Hongwei Wang: Basic Medical College, Medical School of Nanjing University, Nanjing, People’s Republic of China
Bin Cao: Department of Pulmonary and Critical Care Medicine, China–Japan Friendship Hospital, Beijing, People’s Republic of China
Zhiyi He: Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of China

DOI: https://doi.org/10.1080/22221751.2024.2396887
Journal volume & issue: Vol. 13, no. 1

Abstract

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Anti-interferon-γ autoantibodies (AIGAs) syndrome is susceptible to disseminated opportunistic infections due to increased AIGAs, but its clinical immunological characteristics remain unrecognized. We conducted a prospective cohort study between January 2021 and December 2023, recruiting patients with opportunistic infections who were categorized into AIGAs-positive and AIGAs-negative groups. Clinical immunological data and outcomes were documented. A subset of AIGAs-positive patients received glucocorticoid treatment, and its effectiveness was evaluated. A total of 238 patients were enrolled, with 135 AIGAs-positive and 103 AIGAs-negative patients. AIGAs-positive patients showed higher rates of multiple pathogen dissemination, shorter progression-free survival (PFS), and increased exacerbation frequency. They also showed elevated erythrocyte sedimentation rate (ESR), globulin (GLB), immunoglobulin (Ig)G, IgE, and IgG4 levels. Among the 70 AIGAs-positive patients monitored for at least six months, three subtypes were identified: high AIGAs titer with immune damage, high AIGAs titer without immune damage, and low AIGAs titer without immune damage. Of the 55 patients followed for 1 year, decreasing AIGAs titer and immune indices (GLB, IgG, IgE, IgG4) were observed. Among the 31 patients with high AIGAs titer and immune damage treated with low-dose glucocorticoids at the stable phase, reductions were observed in immune indices and AIGAs titer in 67.74% of cases. In summary, AIGAs-positive patients exhibit infectious and immunological characteristics. Elevated AIGAs, IgG, IgG4, and IgE indicate abnormal immune damages. AIGAs titer generally decrease over time. Stable-phase AIGAs-positive patients can be categorized into three subtypes, with those having high AIGAs titer and increased immune indices potentially benefitting from glucocorticoid treatment.

Published in Emerging Microbes and Infections

ISSN: 2222-1751 (Online)
Publisher: Taylor & Francis Group
Country of publisher: United Kingdom
LCC subjects: Medicine: Internal medicine: Infectious and parasitic diseases; Science: Microbiology
Website: https://www.tandfonline.com/journals/temi

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