Tracheobronchial infectious diseases in adult-onset immunodeficiency with anti-interferon-gamma autoantibodies.

Background: Anti-interferon (IFN)-γ autoantibodies predispose subjects to infections caused by opportunistic intracellular pathogens and lead to the involvement of multiple well-recognized organs, such as the lungs, skin, and lymph nodes. However, the clinical characteristics of tracheobronchial involvement remain unrecognized.

Methods: A retrospective study was conducted between February 2016 and September 2024, enrolling patients with anti-IFN-γ autoantibodies who had documented evidence of tracheobronchial involvement. The clinical data were analyzed, including demographic information, clinical manifestations, laboratory results, chest computed tomography images, bronchoscopy findings, treatments, and clinical outcomes.

Results: A total of 33 patients were enrolled, with a mean age of 51.73 years old. The top five reported symptoms were cough, expectoration, fever, dyspnea, and fatigue. Laboratory tests showed elevated white blood cells, C-reactive protein, erythrocyte sedimentation rate, globulin, and immunoglobulin G. The detected pathogens included commonly reported opportunistic pathogens, such as Talaromyces marneffei (57.58%) and nontuberculous mycobacteria (42.42%), as well as the conventional pathogens typically found in patients without immunodeficiency diseases. Masses, nodules, and tracheobronchial stenosis were commonly seen on chest computed tomography scans and during bronchoscopy, directly indicating airway involvement. The predominant pathological results of the tracheobronchial lesions were chronic inflammation, with a small subset showing granuloma formation and abscess formation. Twenty-six patients (78.79%) achieved clinical improvement. Adverse events related to the therapeutic drug were reported in 30.3% of patients, with no life-threatening events.

Conclusions: Tracheobronchial lesions are characterized by masses, nodules, and tracheobronchial stenosis visualized during bronchoscopy, as well as inflammatory changes confirmed by pathologic examinations. Clinicians should be vigilant for tracheobronchial infections, particularly those caused by Talaromyces marneffei and nontuberculous mycobacteria. Pathogen-targeted therapy shows therapeutic benefits but requires close monitoring for drug-related toxicities.