Non-infectious complication of inborn errors of immunity

Published: January 11, 2022

Non-infectious manifestations, i.e. allergy, autoimmunity/inflammation, lymphoproliferation and malignancies are known to be observed in many inborn errors of immunity and to participate to their prognosis. In order to have a global view on their occurrence, the study by Alligon M. et al is based on data collection from a retrospective cohort of 1375 patients included in a national registry for whom a 10-year follow-up since inclusion in the registry is available.

Autoimmune/inflammatory events and malignancies affected all age classes and virtually all diagnostic groups. They were associated with a risk of death that occurred in 14.2% of them. Malignancies and autoimmunity were thus clearly identified as risk factors for lethality. This study points to the frequency and severity of non-infectious manifestations in various groups of inborn errors of immunity across all age groups. These results should be taken into consideration for treatment adaptation and notably indication of curative therapy.

The Journal of Allergy and Clinical Immunology (JACI) is an official scientific journal of the AAAAI, and is the most-cited journal in the field of allergy and clinical immunology.

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