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Review
. 2016 Nov-Dec;4(6):1089-1100.
doi: 10.1016/j.jaip.2016.08.004.

Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies

Jolan E Walter  1 , Jocelyn R Farmer  2 , Zsofia Foldvari  3 , Troy R Torgerson  4 , Megan A Cooper  5
Affiliations
Review

Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies

Jolan E Walter et al. J Allergy Clin Immunol Pract. 2016 Nov-Dec.

Abstract

A broad spectrum of autoimmunity is now well described in patients with primary immunodeficiencies (PIDs). Management of autoimmune disease in the background of PID is particularly challenging given the seemingly discordant goals of immune support and immune suppression. Our growing ability to define the molecular underpinnings of immune dysregulation has facilitated novel targeted therapeutics. This review focuses on mechanism-based treatment strategies for the most common autoimmune and inflammatory complications of PID including autoimmune cytopenias, rheumatologic disease, and gastrointestinal disease. We aim to provide guidance regarding the rational use of these agents in the complex PID patient population.

Keywords: Arthritis; Autoimmune enteropathy (AIE); Autoimmunity; Cytopenias; Inflammatory bowel disease (IBD); Lupus; Primary immunodeficiencies (PIDs); Treatment; Vasculitis.

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Figure 1
Figure 1
Mechanisms of targeting B cell pathology in the treatment of autoimmune and inflammatory diseases associated with PID.
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