Abstract
BackgroundClinical courses and treatment outcomes are largely unknown in patients with adult-onset immunodeficiency associated with anti-interferon-gamma autoantibodies due to the fact that it was recently recognized and anti-IFN-γ auto-Abs detection is not widely available.Methods and findingsNon-HIV-infected adult patients with detectable anti-IFN-γ auto-Abs diagnosed and followed at Siriraj Hospital, Bangkok, Thailand during January 2013 to November 2016 were prospectively studied. At each follow-up visit, patients were classified as stable or active disease according to symptoms and signs, and all proven OIs were recorded. Laboratory parameters, including erythrocyte sedimentation rate, C-reactive protein, and anti-IFN-γ auto-Abs level, were compared between active and stable disease episodes. We identified 80 patients with this clinical syndrome and followed them up during study period. Seventy-nine patients developed overall 194 proven opportunistic infections. Mycobacterium abscessus (34.5%) and Salmonella spp. (23.2%) were the two most common pathogens identified among these patients. Sixty-three patients were followed for a median of 2.7 years (range 0.6–4.8 years). Eleven (17.5%) patients achieved the drug-free remission period for at least 9 months. Four patients died. Anti-IFN-γ auto-Abs concentration was significantly lower at baseline and decreased over time in the drug-free remission group compared to another group (p = 0.001). C-reactive protein, erythrocyte sedimentation rate and white cell count were found to be useful biomarkers for determining disease activity during follow-up.ConclusionsReinfection or relapse of OIs is common despite long-term antimicrobial treatment in patients with anti-IFN-γ auto-Abs. Treatment to modify anti-IFN-γ auto-Abs production may improve long-term outcomes in this patient population.
Highlights
Adult-onset immunodeficiency due to anti-interferon-gamma autoantibodies is a distinct clinical syndrome of disseminated nontuberculous mycobacteria (NTM) or other opportunistic (OI) infections in previously healthy adult patients in association with anti-IFN- γ auto-Abs [1]
Reinfection or relapse of OIs is common despite long-term antimicrobial treatment in patients with anti-IFN-γ auto-Abs
Additional abnormal immune responses in adult-onset immunodeficiency characterized by reduced production of interleukin (IL)-2 and tumor necrosis factor (TNF)-α may be a consequence of T cell exhaustion following chronic antigenic stimulation [11]
Summary
Adult-onset immunodeficiency due to anti-interferon-gamma autoantibodies (anti-IFN-γ auto-Abs) is a distinct clinical syndrome of disseminated nontuberculous mycobacteria (NTM) or other opportunistic (OI) infections in previously healthy adult patients in association with anti-IFN- γ auto-Abs [1]. Patients with antiIFN-γ auto-Abs typically present with disseminated infection caused by opportunistic intracellular pathogens, especially NTM [1, 12,13,14]. A significant proportion of patients are coinfected or present with other intracellular pathogens, including nontyphoidal Salmonella spp., Cryptococcus neoformans, Talaromyces marneffei, and Varicella zoster virus (VZV) [1, 12]. Clinical courses and treatment outcomes are largely unknown in patients with adult-onset immunodeficiency associated with anti-interferon-gamma autoantibodies due to the fact that it was recently recognized and anti-IFN-γ auto-Abs detection is not widely available
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