Clinical immunological characteristics of anti-interferon-γ autoantibodies syndrome: a 3 year prospective cohort study
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.
Authors
- Jie Huang (ORCID: https://orcid.org/0000-0003-4535-4489)
- Jieping Lei (ORCID: https://orcid.org/0000-0002-2862-7249)
- Zhiyi He (ORCID: https://orcid.org/0000-0002-2953-0772)
- Siqiao Liang
- Zhen Wei (ORCID: https://orcid.org/0000-0002-7211-5355)
- Siyao Wu (ORCID: https://orcid.org/0000-0002-1620-9832)
- Rong Xiao (ORCID: https://orcid.org/0000-0002-6214-8057)
- Jingmin Deng (ORCID: https://orcid.org/0000-0003-0189-5371)
- Xin-Xin Zhong
- Chenlu Yang (ORCID: https://orcid.org/0000-0002-0131-6475)
- Honglin Luo (ORCID: https://orcid.org/0000-0002-7708-7840)
- Jiujin Zhang
- Hanlin Liang (ORCID: https://orcid.org/0000-0001-9662-8246)
- Bin Cao (ORCID: https://orcid.org/0000-0001-6991-0350)
- Meihua Li (ORCID: https://orcid.org/0000-0003-1184-5482)
- Pei Li (ORCID: https://orcid.org/0000-0002-2870-7484)
- Xiaokai Feng
- Meiling Yang (ORCID: https://orcid.org/0000-0002-9690-5458)
- Ke Wang (ORCID: https://orcid.org/0000-0001-9457-7166)
- Wen Zeng (ORCID: https://orcid.org/0000-0003-2471-3235)
- Jing Jiang (ORCID: https://orcid.org/0000-0002-1943-6010)
- Shouming Qin (ORCID: https://orcid.org/0000-0002-7411-0101)
- Haijuan Tang (ORCID: https://orcid.org/0000-0002-1780-7607)
- Xuemei Huang (ORCID: https://orcid.org/0000-0002-1883-9731)
- Yan Ning
- Jian Song (ORCID: https://orcid.org/0000-0002-2459-7046)
- Ni Chen
- Xiaona Liang
- Zengtao Luo
- Chengqiong Xu
- Fu Cao
- Quanfang Chen
- Qing Wei
- Hongwei Wang (ORCID: https://orcid.org/0009-0005-4294-1102)
Institutions
- Guangxi Medical University (CN)
- Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
- Beijing Chao-Yang Hospital, Capital Medical University (CN)
- China-Japan Friendship Hospital (CN)
- The Fourth People's Hospital (CN)
- Qinhuangdao Second Hospital (CN)
- Kingmed Diagnostics (CN)
- The People's Hospital of Guangxi Zhuang Autonomous Region (CN)
- First Affiliated Hospital of GuangXi Medical University (CN)
- Guangxi Academy of Sciences (CN)
- Nanjing Medical University (CN)
- Nanjing University (CN)
Publication Details
- Journal
- Emerging Microbes & Infections
- Published
- 2024-08-23
- DOI
- https://doi.org/10.1080/22221751.2024.2396887
- Citations
- 10
- Primary Topic
- Immunodeficiency and Autoimmune Disorders
- Type
- article
- Field-Weighted Citation Impact
- 2.40