James W Verbsky1, Talal A Chatila. 1. Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. jverbsky@mcw.edu
Abstract
PURPOSE OF REVIEW: To summarize studies on the development and function of T-regulatory (TR) cells in primary immune deficiencies (PIDs). RECENT FINDINGS: PIDs are associated with high rates of autoimmunity. TR cells, which are critical to the control of autoimmunity, appear involved in the pathogenesis of PID-related autoimmunity. A number of PIDs, including Omenn's syndrome and Wiskott-Aldrich syndrome, have been associated with impaired production and/or function of thymus-derived (natural) TR cells. Recently defined primary immunodeficiencies, including Stim1 deficiency, IL-10 receptor deficiency, and xIAP deficiency, have been associated with defects in TR cells. De-novo generated TR cells from peripheral CD4 conventional T cells is impaired in the hyper IgE syndrome. SUMMARY: Gene defects underlying PIDs may also compromise the TR cell, leading to breakdown of peripheral tolerance.
PURPOSE OF REVIEW: To summarize studies on the development and function of T-regulatory (TR) cells in primary immune deficiencies (PIDs). RECENT FINDINGS: PIDs are associated with high rates of autoimmunity. TR cells, which are critical to the control of autoimmunity, appear involved in the pathogenesis of PID-related autoimmunity. A number of PIDs, including Omenn's syndrome and Wiskott-Aldrich syndrome, have been associated with impaired production and/or function of thymus-derived (natural) TR cells. Recently defined primary immunodeficiencies, including Stim1 deficiency, IL-10 receptor deficiency, and xIAP deficiency, have been associated with defects in TR cells. De-novo generated TR cells from peripheral CD4 conventional T cells is impaired in the hyper IgE syndrome. SUMMARY: Gene defects underlying PIDs may also compromise the TR cell, leading to breakdown of peripheral tolerance.
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