Clinical Cognition and Practice in Anti-Interferon-γ Autoantibody-Associated Immunodeficiency Syndrome - PubMed
5 hours ago
- #Immunodeficiency
- #Opportunistic Infections
- #Autoantibody
- Adult-onset immunodeficiency syndrome mediated by anti-interferon-gamma autoantibody (AIGA) is a rare disorder increasingly recognized, especially prevalent in Southeast Asia.
- It causes recurrent disseminated opportunistic infections affecting multiple organ systems like lungs, lymph nodes, bones, and skin due to impaired IFN-γ signaling.
- Diagnosis involves quantifying AIGA titers and assessing neutralizing activity, supported by clinical manifestations and seropositivity.
- Management includes antimicrobial therapy in acute stages and categorizes stable cases into three subtypes based on AIGA titers and immune damage.
- Patients with high AIGA titers and immune damage require immunomodulatory strategies, such as glucocorticoids and rituximab, tailored to individual status.
- The review synthesizes evidence on epidemiology, pathogenesis, and treatment to aid clinicians and encourage further research and guidelines.