Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies.
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.
Department of Pediatrics & Medicine, University of South Florida at Johns Hopkins All Children's Hospital, St Petersburg, Fla; Division of Pediatric Allergy & Immunology, Massachusetts General Hospital for Children, Boston, Mass; Division of Immunology, Boston Children's Hospital, Boston, Mass. Electronic address: jolanwalter@health.usf.edu.
Jolan E
JE
Walter
Department of Allergy & Immunology, Massachusetts General Hospital, Boston, Mass.
Jocelyn R
JR
Farmer
Department of Cancer Immunology, Oslo University Hospital Radiumhospitalet, Oslo, Norway; K. G. Jebsen Centers for Cancer Immunotherapy and for Inflammation Research, Institute for Clinical Medicine, University of Oslo, Oslo, Norway.
Zsofia
Z
Foldvari
Department of Pediatrics, University of Washington School of Medicine, Seattle, Wash.
Troy R
TR
Torgerson
Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, St Louis, Mo.
Megan A
MA
Cooper
D000818:Animals; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D015551:Autoimmunity; D005767:Gastrointestinal Diseases / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000188:drug therapy* / Q000276:immunology
Arthritis; Autoimmune enteropathy (AIE); Autoimmunity; Cytopenias; Inflammatory bowel disease (IBD); Lupus; Primary immunodeficiencies (PIDs); Treatment; Vasculitis