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

Jolan E. Walter; Jocelyn R. Farmer; Zsofia Foldvari; Troy R. Torgerson; Megan A. Cooper

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, Jocelyn R. Farmer, Zsofia Foldvari, Troy R. Torgerson, Megan A. Cooper

Research output: Contribution to journal › Review article › peer-review

65 Scopus citations

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.

Original language	English
Pages (from-to)	1089-1100
Number of pages	12
Journal	Journal of Allergy and Clinical Immunology: In Practice
Volume	4
Issue number	6
DOIs	https://doi.org/10.1016/j.jaip.2016.08.004
State	Published - Nov 1 2016

Keywords

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

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10.1016/j.jaip.2016.08.004

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title = "Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies",

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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AU - Walter, Jolan E.

AU - Farmer, Jocelyn R.

AU - Foldvari, Zsofia

AU - Torgerson, Troy R.

AU - Cooper, Megan A.

PY - 2016/11/1

Y1 - 2016/11/1

N2 - 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.

AB - 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.

KW - Arthritis

KW - Autoimmune enteropathy (AIE)

KW - Autoimmunity

KW - Cytopenias

KW - Inflammatory bowel disease (IBD)

KW - Lupus

KW - Primary immunodeficiencies (PIDs)

KW - Treatment

KW - Vasculitis

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DO - 10.1016/j.jaip.2016.08.004

M3 - Review article

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SN - 2213-2198

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SP - 1089

EP - 1100

JO - Journal of Allergy and Clinical Immunology: In Practice

JF - Journal of Allergy and Clinical Immunology: In Practice

IS - 6

ER -

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

Abstract

Keywords

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