• Xiao-Na Liang, Yan-Fei Bin, Guan-Ting Lai, Ying-Hua Li, Jian-Quan Zhang, Xiao-Ning Zhong, Jing Bai, Mei-Hua Li, Jing-Min Deng, and Zhi-Yi He.
• Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Guangxi Zhuang Autonomous Region, P.R. China.
• Medicine (Baltimore). 2020 Sep 4; 99 (36): e21738.

IntroductionAnti-interferon-gamma (anti-IFN-γ) autoantibody increases susceptibility to lower-virulence pathogens and causes immunodeficiency syndrome in HIV-negative patients.Patient ConcernsA 69-year-old Chinese man presented with a 2-month history of pruritic skin lesions on his forearms, trunk, and legs. He was diagnosed with 5 opportunistic infections without conventional immunosuppression-associated factors in past. The most conspicuous characteristics were recurrent pulmonary infection, persistent immunoglobulin E elevation and eosinophilia during the whole disease course.DiagnosisEnzyme-linked immunosorbent assay showed anti-IFN-γ autoantibody positive. The final diagnosis for the patient was adult-onset immunodeficiency due to anti-IFN-γ autoantibody, non-tuberculous mycobacterial (NTM) infection and reactive dermatosis.InterventionsThe patient underwent long-term anti-NTM and corticosteroid maintenance treatment.OutcomesThe patient was followed for 2 years during which opportunistic infection no longer happened, the immunoglobulin E level and eosinophil count reduced, the autoantibody levels remained largely steady and lung lesions absorbed.ConclusionClinicians should be vigilant for NTM infection in patients with anti-IFN-γ autoantibodies, even when culture results are negative. Long-term anti-non-tuberculous mycobacteria and glucocorticoid regimens were effective.

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