文摘
Primary immunodeficiency disorders (PID) are frequently complicated by autoimmune and inflammatory conditions. Disease of immune dysregulation have improved our knowledge of the pathways that drive autoimmunity in PIDs. A number of heritable immune dysregulatory diseases arise from defects which affect Treg cell development, function, or both. These consist of immune dysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX) and IPEX-like disorders.