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

J Allergy Clin Immunol Pract. 2016 Nov-Dec;4(6):1089-1100. doi: 10.1016/j.jaip.2016.08.004.

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.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Autoimmune Diseases / drug therapy*
  • Autoimmune Diseases / etiology
  • Autoimmune Diseases / immunology
  • Autoimmunity
  • Gastrointestinal Diseases / drug therapy*
  • Gastrointestinal Diseases / etiology
  • Gastrointestinal Diseases / immunology
  • Humans
  • Immunologic Deficiency Syndromes / complications
  • Immunologic Deficiency Syndromes / drug therapy*
  • Immunologic Deficiency Syndromes / immunology