Autoimmune and inflammatory manifestations occur frequently in primary immunodeficiencies

Published online: February 10, 2017

Primary immunodeficiencies (PID) is a group of inherited diseases with an overall incidence estimated to be of 1 in 3000 to 4000 live births. In addition to frequent infections, PID are associated with non-infectious complications including allergy, autoimmunity and inflammation but there are very few studies about the incidence of the non-allergic and non-infectious complications.

In a recent study published in  The Journal of Allergy and Clinical Immunology (JACI) Fischer and colleagues examined a retrospective analysis of 2183 patient with PID that have been reported to the French national registry.

Autoimmunity and/or inflammation were detected in 26 % of these patients. These were identified in children and adults leading to a risk of approximately 40 % by the age of 50 years. Although the risk varies as a function of the specific PID disease, it appears the risk increases in virtually all of PID categories. This study opens the way to future work aimed at understanding the mechanism by which PID predispose to autoimmunity and inflammation.

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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