Abstract
Primary immunodeficiency disorders (PIDs) are often associated with autoimmune disease due to the dysregulation of the immune system and distributed central and peripheral tolerance. In many inborn immune deficiencies, lymphocytes may be present but they are dysfunctional, allowing for the development of excessive autoreactivity skipping negative selection process and resultant autoimmune disease. This chapter summarizes the clinical approaches to the main immune dysregulations including hemophagocytic lymphohistiocytosis syndromes, autoimmunity with lymphoproliferation and autoimmunity without lymphoproliferation.
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