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 journalReview articlepeer-review

50 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 languageEnglish
Pages (from-to)1089-1100
Number of pages12
JournalJournal of Allergy and Clinical Immunology: In Practice
Volume4
Issue number6
DOIs
StatePublished - Nov 1 2016

Keywords

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

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