Immune Dysregulation in Primary Immunodeficiency Disorders

The past several years have brought an increased awareness of the prevalence of autoimmunity and immune dysregulation among patients who have primary immunodeficiency disorders (PIDD). The recent clinical and molecular definition of PIDD, in which the primary defect is in the immunoregulatory compartment of the immune system, has offered insight into the basic mechanisms of immune tolerance, which has provided new targets and new techniques to study immune tolerance in PIDD. Many of these studies have focused on the presence and function of regulatory T (TREG) cells in PIDD, particularly since the discovery of murine and human syndromes associated with TREG deficiency. This article focuses on the current state of knowledge regarding the role of TREG in various PIDD that have clinical features indicative of dysregulated immunity.