Nontuberculous mycobacteria (NTM) infections involving anti-interferon-gamma(IFN-gamma)-neutralizing auto antibodies have been described in previously immunocompetent adults. To investigate the factors underlying various disease manifestations, we reviewed 35 articles published between January 2004 and November 2016 and identified 111 NTM patients with anti-IFN-gamma, autoantibodies. Rapidly growing mycobacteria (RGM) accounted for 53% of the isolated species. RGM were predominant among the NTM species isolated from Thai (73%), Chinese (58%) and Filipino (56%) patients, whereas M. avium complex (MAC) was predominant among Japanese (58%) and non-Asian (80%) patients. The commonly involved organs included the lymph nodes (79%), bones/joints (34%) and lungs (32%). Compared with the patients with MAC, the patients with RGM had a higher incidence of lymph node lesions (P < 0.05) and a lower incidence of bone/joint (P < 0.01), lung (P < 0.01), soft tissue (P < 0.01), bronchus (P < 0.01) and muscle (P < 0.05) lesions. Clinical manifestations of NTM disease with anti-IFN-gamma-neutralizing autoantibodies differ across ethnicities and NTM species. (C) 2017 Elsevier Inc. All rights reserved.