Factors associated with and kinetics of anti-IFN-α autoantibodies in <i>RAG1/2</i> deficiency.

ABSTRACT:

Background

Autoantibodies against IFN-α (anti-IFN-α) have been reported in recombinase activating gene (RAG) deficiency, attributed to impaired central and peripheral T-cell/B-cell tolerance. However, the clinical features, especially viral infections, associated with these autoantibodies at baseline, their kinetics over time, and their response to hematopoietic cell transplantation are not well defined.

Objective

We described the clinical and immunologic findings linked to anti-IFN-α IgG in RAG deficiency and tracked its kinetics longitudinally, including in those who underwent hematopoietic cell transplantation.

Methods

We measured anti-IFN-α IgG by enzyme-linked immunosorbent assay in 80 RAG-deficient patients with curated clinical and immunologic data from a multi

SUBMITTER: Wang C 

PROVIDER: S-EPMC12281840 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature