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Monogenic Polyautoimmunity in Primary Immunodeficiency Diseases Publisher Pubmed



Azizi G1, 2, 3 ; Yazdani R2 ; Rae W4 ; Abolhassani H2, 5 ; Rojas M6 ; Aghamohammadi A2, 7 ; Anaya JM6
Authors

Source: Autoimmunity Reviews Published:2018


Abstract

Primary immunodeficiency diseases (PIDs) consist of a large group of genetic disorders that affect distinct components of the immune system. PID patients are susceptible to infection and non-infectious complications, particularly autoimmunity. A specific group of monogenic PIDs are due to mutations in genes that are critical for the regulation of immunological tolerance and immune responses. This group of monogenic PIDs is at high risk of developing polyautoimmunity (i.e., the presence of more than one autoimmune disease in a single patient) because of their impaired immunity. In this review, we discuss the mechanisms of autoimmunity in PIDs and the characteristics of polyautoimmunity in the following PIDs: IPEX; monogenic IPEX-like syndrome; LRBA deficiency; CTLA4 deficiency; APECED; ALPS; and PKCδ deficiency. © 2018 Elsevier B.V.
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