Published online Nov 16, 2023. doi: 10.12998/wjcc.v11.i32.7926
Peer-review started: September 26, 2023
First decision: October 9, 2023
Revised: October 23, 2023
Accepted: November 9, 2023
Article in press: November 9, 2023
Published online: November 16, 2023
Several reports of adult-onset immunodeficiency syndrome have been associated with anti-interferon-gamma (IFN-γ) autoantibodies (AIGAs). However, it is rare to find AIGAs with intracranial infections.
In this case study, we report a case of an AIGAs with intracranial infection and hand rashes considered Sweet’s syndrome. The patient presented to our hospital with a persistent cough, a fever that had been going on for 6 mo, and a rash that had been going on for a week. The patient started losing consciousness gradually on the fourth day after admission, with neck stiffness and weakened limb muscles. The upper lobe of the left lung had a high-density mass with no atypia and a few inflammatory cells in the interstitium. Brain magnetic resonance imaging and cerebrospinal fluid suggest intracranial infection. The pathology of the skin damage on the right upper extremity revealed an infectious lesion that was susceptible to Sweet’s disease. It has an anti-IFN-γ autoantibody titer of 1:2500. She was given empirical anti-non-tuberculous mycobacterial and anti
Adults with severe and recurrent infections of several organs should be considered for AIGAs if no other known risk factors exist. AIGAs are susceptible to subsequent intracranial infections and Sweet’s syndrome.
Core Tip: Anti-interferon-gamma (IFN-γ) autoantibodies (AIGAs) have been associated with adult-onset immunodeficiency syndrome. Most patients have multiple organ involvement upon presentation; lymph nodes are the most frequently affected organ, followed by the skin, lungs, bones, and joints. We describe a patient with AIGAs who also had an intracranial infection and hand rashes as Sweet’s syndrome. Anti-IFN-γ was increased despite the lack of a confirmed non-tuberculous mycobacterial (NTM) infection, and the empirical anti-NTM treatment in this patient was successful. Without recognized risk factors, AIGAs should be considered in patients with severe and recurrent infections of multiple organs. In AIGAs, subsequent intracranial infection and Sweet’s syndrome are possible.