Ning Yan, Liang Hanlin, Liang Siqiao, Liang Xiaona, Huang Xuemei, He Zhiyi
Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, No.6 Shuang Yong Road, Nanning, Guangxi, 530021, China.
BMC Neurol. 2025 Aug 25;25(1):345. doi: 10.1186/s12883-025-04380-5.
Recently, increased reports reveal that anti-interferon-gamma (IFN-γ) autoantibodies (AIGAs) are strongly associated with several severe disseminated infections. However, reports on AIGAs with central nervous system (CNS) infections are rare. Here, we described three AIGAs-positive adults who had persistent or recurrent disseminated infections caused by Talaromyces marneffei (TM), nontuberculous mycobacteria (NTM), mycobacterium tuberculosis (TB), or other pathogens, accompanied with CNS infections. In addition, we conducted a thorough literature review of AIGAs-positive patients with CNS infections.
We report three HIV-negative cases of recurrent disseminated infections including CNS, and AIGAs were measured. All patients had no history of underlying diseases or immunosuppression and presented with fever, cough, and headache. They were negative for HIV antibodies but positive for AIGAs. The patients were diagnosed with CNS infections based on cerebrospinal fluid (CSF) examination and next-generation sequencing (NGS). All patients received anti-infective treatment according to different pathogens, and their condition remained stable without recurrence.
In adults with severe and recurrent infections of multiple organs without known immunodeficiency, adult-onset immunodeficiency (AOID) associated with AIGAs should be considered. In AIGAs-positive patients, the blood-brain barrier (BBB) may be disrupted, leading to susceptibility to CNS infections.
最近，越来越多的报告显示，抗干扰素-γ（IFN-γ）自身抗体（AIGAs）与几种严重的播散性感染密切相关。然而，关于AIGAs与中枢神经系统（CNS）感染的报道却很少。在此，我们描述了三名AIGAs阳性的成年人，他们患有由马尔尼菲篮状菌（TM）、非结核分枝杆菌（NTM）、结核分枝杆菌（TB）或其他病原体引起的持续性或复发性播散性感染，并伴有CNS感染。此外，我们对AIGAs阳性的CNS感染患者进行了全面的文献综述。
我们报告了三例HIV阴性的复发性播散性感染病例，包括CNS感染，并检测了AIGAs。所有患者均无基础疾病或免疫抑制病史，表现为发热、咳嗽和头痛。他们的HIV抗体检测为阴性，但AIGAs检测为阳性。根据脑脊液（CSF）检查和二代测序（NGS），这些患者被诊断为CNS感染。所有患者根据不同病原体接受了抗感染治疗，病情保持稳定，无复发。
对于无已知免疫缺陷的严重复发性多器官感染的成年人，应考虑与AIGAs相关的成人起病免疫缺陷（AOID）。在AIGAs阳性患者中，血脑屏障（BBB）可能被破坏，导致易患CNS感染。
