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已发表论文
严重沙门氏菌感染在 AIGA 免疫缺陷综合征中的表现：过度炎症与免疫失调
Authors Liang S, Chen F, Huang X, Ning Y, Wu S , Hong L, Chen N, Liang H, Wang M, Liang X, He Z
Received 13 June 2025
Accepted for publication 5 December 2025
Published 8 January 2026 Volume 2026:19 546839
DOI https://doi.org/10.2147/IDR.S546839
Checked for plagiarism Yes
Review by Single anonymous peer review
Peer reviewer comments 2
Editor who approved publication: Dr Hemant Joshi
Siqiao Liang,1,* Fushou Chen,2,* Xuemei Huang,1 Yan Ning,1 Siyao Wu,1 Limei Hong,1 Ni Chen,1 Hanlin Liang,1 Mengchan Wang,1 Xiaona Liang,1 Zhiyi He1
1Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, People’s Republic of China; 2Department of Respiratory and Critical Care Medicine, Wuming Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, People’s Republic of China
*These authors contributed equally to this work
Correspondence: Zhiyi He, Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuang Yong Road, Nanning, Guangxi, 530021, People’s Republic of China, Email zhiyi-river@163.com Xiaona Liang, Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuang Yong Road, Nanning, Guangxi, 530021, People’s Republic of China, Email xnliang0318@163.com
Background: Anti-interferon-γ autoantibodies (AIGAs) immunodeficiency syndrome is a rare acquired disorder characterized by impaired IFN-γ signaling, predisposing patients to severe intracellular infections. While disseminated non-tuberculous mycobacteria (NTM) and Talaromyces marneffei (TM) are well-documented pathogens, the clinical and immunological features of Salmonella coinfection remain poorly characterized.
Methods: This retrospective study analyzed 12 HIV-negative patients with AIGAs-positive status and confirmed Salmonella infection at the First Affiliated Hospital of Guangxi Medical University, China (2021– 2024). Data included demographics, clinical manifestations, laboratory findings, co-infections, treatment, and outcomes. AIGAs were detected via ELISA and Western blot, with neutralizing activity confirmed by STAT1 phosphorylation inhibition.
Results: The cohort was predominantly composed of middle-aged males (83.3%, mean age 55.75 ± 8.06 years). The most common symptoms were fever, fatigue and cough (each 91.7%), followed by poor appetite (83.3%), systemic symptoms (chills, weight loss; 58.3%) and dyspnea (58.3%). Bone or joint pain occurred in 41.7% and gastrointestinal complaints (abdominal pain, diarrhea or distension) in 25%. Five patients (41.7%) developed septic shock, three requiring vasopressors and two mechanical ventilations. All had high AIGAs titres (1:2500) and hyper-inflammation (median WBC17.3× 109/L, CRP138.1mg/dL, PCT1.28ng/mL). Bacteraemia was present in 91.7% and mortality was 16.7% (2/12). Polymicrobial co-infection was universal; notably cytomegalovirus (50%) and TM (25%). Immunological profiling showed hyperglobulinaemia (IgG23.5± 10.6g/L) and elevated IgE (257.5[79.7– 598.2]IU/mL). Despite broad-spectrum antibiotics (83.3% survival), both fatalities occurred in patients who had not undergone NGS-based diagnosis.
Conclusion: This study is the first to define AIGAs-associated Salmonella infection as a distinct clinical syndrome, characterized by severe bacteremia, paradoxical hyperinflammation, universal polymicrobial coinfections, and immune dysregulation. Our findings underscore the critical importance of comprehensive pathogen detection, particularly via NGS, for timely diagnosis and improved patient outcomes.
Keywords: anti-interferon-γ autoantibodies, Salmonella, hyperinflammation, opportunistic infections, immunodeficiency
1Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, People’s Republic of China; 2Department of Respiratory and Critical Care Medicine, Wuming Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, People’s Republic of China
*These authors contributed equally to this work
Correspondence: Zhiyi He, Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuang Yong Road, Nanning, Guangxi, 530021, People’s Republic of China, Email zhiyi-river@163.com Xiaona Liang, Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, No. 6 Shuang Yong Road, Nanning, Guangxi, 530021, People’s Republic of China, Email xnliang0318@163.com
Background: Anti-interferon-γ autoantibodies (AIGAs) immunodeficiency syndrome is a rare acquired disorder characterized by impaired IFN-γ signaling, predisposing patients to severe intracellular infections. While disseminated non-tuberculous mycobacteria (NTM) and Talaromyces marneffei (TM) are well-documented pathogens, the clinical and immunological features of Salmonella coinfection remain poorly characterized.
Methods: This retrospective study analyzed 12 HIV-negative patients with AIGAs-positive status and confirmed Salmonella infection at the First Affiliated Hospital of Guangxi Medical University, China (2021– 2024). Data included demographics, clinical manifestations, laboratory findings, co-infections, treatment, and outcomes. AIGAs were detected via ELISA and Western blot, with neutralizing activity confirmed by STAT1 phosphorylation inhibition.
Results: The cohort was predominantly composed of middle-aged males (83.3%, mean age 55.75 ± 8.06 years). The most common symptoms were fever, fatigue and cough (each 91.7%), followed by poor appetite (83.3%), systemic symptoms (chills, weight loss; 58.3%) and dyspnea (58.3%). Bone or joint pain occurred in 41.7% and gastrointestinal complaints (abdominal pain, diarrhea or distension) in 25%. Five patients (41.7%) developed septic shock, three requiring vasopressors and two mechanical ventilations. All had high AIGAs titres (1:2500) and hyper-inflammation (median WBC17.3× 109/L, CRP138.1mg/dL, PCT1.28ng/mL). Bacteraemia was present in 91.7% and mortality was 16.7% (2/12). Polymicrobial co-infection was universal; notably cytomegalovirus (50%) and TM (25%). Immunological profiling showed hyperglobulinaemia (IgG23.5± 10.6g/L) and elevated IgE (257.5[79.7– 598.2]IU/mL). Despite broad-spectrum antibiotics (83.3% survival), both fatalities occurred in patients who had not undergone NGS-based diagnosis.
Conclusion: This study is the first to define AIGAs-associated Salmonella infection as a distinct clinical syndrome, characterized by severe bacteremia, paradoxical hyperinflammation, universal polymicrobial coinfections, and immune dysregulation. Our findings underscore the critical importance of comprehensive pathogen detection, particularly via NGS, for timely diagnosis and improved patient outcomes.
Keywords: anti-interferon-γ autoantibodies, Salmonella, hyperinflammation, opportunistic infections, immunodeficiency