Biologics and JAK inhibitors for atopic manifestations in inborn errors of immunity: a systematic review.

Background: Atopy might be the initial or dominant manifestation in patients with inborn errors of immunity (IEIs). Biologics and Janus kinase (JAK) inhibitors have emerged as targeted options for atopic disorders in IEIs refractory to conventional treatment. Methods: We conducted a systematic review of PubMed, Web of Science, and Scopus for human studies reporting biologics or JAK inhibitors used specifically to treat atopic manifestations in IEIs. Data on genotypes, clinical phenotypes, treatments, outcomes, adverse events, and laboratory changes were extracted and synthesized. The databases were last searched in April 2026. Study quality was appraised with the Joanna Briggs Institute checklists and the certainty of evidence was rated following GRADE principles. Results: Eighty-one studies comprising 172 patients were included. The most common IEIs were STAT3 loss-of-function (LOF) disease ( Conclusion: Published case reports and small series suggest that biologics and JAK inhibitors may provide clinically meaningful control of selected atopic or atopic-like manifestations in some patients with IEIs. Dupilumab has the largest published experience, while JAK inhibitors may be particularly relevant when the underlying disorder involves JAK-STAT pathway activation. Due to the lack of evidence, treatment should remain individualized, genotype-informed, and accompanied by careful infection surveillance and longitudinal outcome assessment.