Autoimmune and inflammatory manifestations occur frequently in primary immunodeficiencies

Publication date: Available online 10 February 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Alain Fischer, Johan Provot, Jean-Philippe Jais, Alexandre Alcais, Nizar Mahlaoui
BackgroundPrimary immunodeficiencies (PIDs) are inherited diseases associated with a considerably increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases including allergy, autoimmunity and inflammation.Objective: We aimed at determining the incidence of autoimmunity and inflammation in PID patients.Methods: We have retrospectively screen 2183 consecutive cases of PID in the French CEREDIH registry for the occurrence of autoimmunity and inflammation.Results: One or more autoimmune and inflammatory complications were noted in 26.2% of patients, with a risk of onset throughout the patient's lifetime. The risk of autoimmune cytopenia was at least 120 times higher than in the general population, the risk of inflammatory bowel disease in children 80 times higher and the risk of other autoimmune manifestations was approximately 10 times higher. Remarkably, all types of PID were associated with a risk of autoimmune and inflammatory complications, although the greatest risk was associated with T cell PIDs and common variable immunodeficiency. The occurrence of autoimmune disease is a negative prognostic factor for survival.ConclusionsOur results provide the basis for a detailed, prospective evaluation of autoimmunity and inflammation in the context of PID, with a view to accurately assessing these risks and describing the possible impact of medical intervention.



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