Case report reveals CNS infection in an immunodeficiency syndrome, highlighting screening importance.
Acquired Anti-interferon (IFN)-γ Autoantibody Syndrome (AAS) is an emerging immunodeficiency predisposing to disseminated nontuberculous mycobacterial infections. We report a 51-year-old woman with AAS presenting with Sweet’s syndrome, disseminated Mycobacterium abscessus subsp . bolletii infection, and central nervous system (CNS) involvement manifested as leptomeningeal enhancement despite sterile cerebrospinal fluid. Diagnosis was confirmed by serum anti-IFN-γ autoantibody titer >1:10,000 and lymph node culture. The patient achieved sustained remission through a staged approach: 26 months of tailored antibiotics (imipenem/ceftazidime/amikacin/clarithromycin-based regimen) followed by delayed cyclophosphamide immunotherapy. This case highlights the importance of anti-IFN-γ autoantibody screening in disseminated infections and demonstrates that CNS involvement may occur without typical cerebrospinal fluid abnormalities. Our management strategy-prioritizing infection control before immunosuppression-provides a pragmatic framework for resource-limited settings.
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