TY - JOUR
T1 - Autoimmune disease in primary immunodeficiency
T2 - At the Crossroads of Anti-Infective Immunity and Self-Tolerance
AU - Saifi, Maryam
AU - Wysocki, Christian A.
N1 - Publisher Copyright:
© 2015 Elsevier Inc. All rights reserved.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2015/11/1
Y1 - 2015/11/1
N2 - The association of autoimmunity and primary immunodeficiency suggests the existence of mechanistic links between development of the various elements of the immune system and the maintenance of self-tolerance. In this review, various monogenic primary immunodeficiencies (PID) are systematically explored, with a specific focus on the impact of these genetic lesions on tolerance, correlating these defects in tolerance with clinical autoimmune and inflammatory syndromes seen in these PIDs. Common variable immunodeficiency (CVID) is explored, and areas are highlighted in which findings in monogenic PID are beginning to illuminate the mechanisms behind these conditions in CVID.
AB - The association of autoimmunity and primary immunodeficiency suggests the existence of mechanistic links between development of the various elements of the immune system and the maintenance of self-tolerance. In this review, various monogenic primary immunodeficiencies (PID) are systematically explored, with a specific focus on the impact of these genetic lesions on tolerance, correlating these defects in tolerance with clinical autoimmune and inflammatory syndromes seen in these PIDs. Common variable immunodeficiency (CVID) is explored, and areas are highlighted in which findings in monogenic PID are beginning to illuminate the mechanisms behind these conditions in CVID.
KW - Apoptosis
KW - Autoimmunity
KW - Common variable immunodeficiency
KW - Primary immunodeficiency
KW - Regulatory T cell
KW - Tolerance
UR - http://www.scopus.com/inward/record.url?scp=84955185358&partnerID=8YFLogxK
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U2 - 10.1016/j.iac.2015.07.007
DO - 10.1016/j.iac.2015.07.007
M3 - Review article
C2 - 26454316
AN - SCOPUS:84955185358
SN - 0889-8561
VL - 35
SP - 731
EP - 752
JO - Immunology and Allergy Clinics of North America
JF - Immunology and Allergy Clinics of North America
IS - 4
ER -