Tehran University of Medical Sciences

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Abolhassani H1, 2 ; Dabbaghzadeh A3 ; Ahanchian H4 ; Khalighi N4 ; Ghaffari J5 ; Ehlayel M6 ; Espanol T7 ; Crimi RD7, 8 ; Agullo PL9 ; Niehues T10
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Source: Inborn Errors of Immunity: A Practical Guide Published:2021


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. © 2021 Elsevier Inc. All rights reserved.
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