Abstract
Objective Interferon gamma receptor 1 (IFNGR1) deficiency is a primary immunodeficiency that causes systemic infections with weakly virulent nontuberculous mycobacteria, which are very difficult to control with combined antimycobacterial agents. Methods Disseminated mycobacterial infection that could not be controlled with combined antimycobacterial treatments in a 13-year-old patient with a novel mutation in the IFNGR1 gene. Despite receiving intravenous antibiotic therapy, fever continued, bone involvement gradually increased, and proteinuria and microscopic hematuria occurred. Peg-IFNα-2a was administered as an adjuvant therapy that resulted in good clinical response with no side effects. Fever improved and no new bone lesions occurred with edema of existing lesions regressing after 2 weeks of peg-IFNα-2a therapy. Conclusion Aggressive treatment with antimycobacterial antibiotics and hematopoietic stem cell transplantation remains the single accepted method for the management of patients with complete IFNGR1 deficiency. Peg-IFNα-2a treatment may be an adjunct treatment in this disorder.
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