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

What are the clinical features and management strategies for immunodeficiency associated with anti–interferon-γ autoantibodies (AIGA)?This cohort study of 38 patients with AIGA immunodeficiency, including 21 treated with rituximab in a clinical setting, found sustained infection remission, improved survival, and acceptable safety profiles.Results of this study suggest that this rare immunodeficiency syndrome can be effectively managed with rituximab and monitored by functional Stat1 phosphorylation neutralization assays.

Corresponding Author: Philip Hei Li, MD, Division of Rheumatology and Clinical Immunology, Department of Medicine, The University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Rd, Pokfulam, Hong Kong (liphilip@hku.hk).

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