Prospective Registry of Outcomes, Treatment, and Clinical Trajectories for Anti–IFN-γ Immunodeficiency

Category	Detail
Condition	Anti–Interferon-γ Autoantibody Induced Immunodeficiency
Key Mechanisms	Neutralization of endogenous IFN-γ impairs the IFN-γ–interleukin 12 pathway, compromising macrophage-mediated intracellular killing.
Target Population	Adults, particularly East Asian ethnic groups, with a significant female skew in US cohorts.
Care Setting	Tertiary referral center in Hong Kong with a unified public health care system.

AIGA immunodeficiency is characterized by increased susceptibility to nontuberculous mycobacteria and other opportunistic pathogens.
Diagnosis involves detection of autoantibodies and functional confirmation via Stat1 phosphorylation inhibition.
Rituximab is the most reported treatment, showing clinical improvement in small case series.
The AIGA-PROTECT registry captures comprehensive clinical data for patients with AIGA immunodeficiency.
Delayed diagnosis and treatment are common due to low clinical awareness and limited access to confirmatory assays.

Initial detection of anti–interferon-γ autoantibodies.
Functional confirmation of pathogenicity through Stat1 phosphorylation assessment.

Consider rituximab on a case-by-case basis, guided by clinical severity and infection burden.

Patients diagnosed with AIGA immunodeficiency, primarily adults in East Asia.

Rituximab is used off-label for AIGA immunodeficiency, with treatment decisions based on individual clinical assessments.

Utilize a centralized platform for standardized testing and clinical management.
Increase clinical awareness to reduce delays in diagnosis and treatment.

Clinical Scorecard: Registry of Clinical Outcomes, Treatments, and Progression in Anti–IFN-γ Induced Immunodeficiency