Case Report: Disseminated Mycobacterium abscessus subsp. bolletii infection with central nervous system involvement in acquired anti-IFN-γ autoantibody syndrome

TL;DR

A 51-year-old woman with a rare immune disorder developed a severe mycobacterial infection affecting her nervous system, and was successfully treated with a staged antibiotic and immunotherapy approach.

Contribution

This case report presents a novel management strategy for AAS with CNS involvement using a staged treatment approach in resource-limited settings.

Findings

CNS involvement in AAS can occur without typical cerebrospinal fluid abnormalities.

A staged treatment approach with antibiotics followed by immunotherapy achieved sustained remission.

Anti-IFN-γ autoantibody screening is crucial in cases of disseminated mycobacterial infections.

Abstract

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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