The Journal of Allergy and Clinical Immunology: In Practice
Volume 4, Issue 6, November–December 2016, Pages 1089-1100
Review and Theme ArticleMechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies
Under a Creative Commons license
open access
Key words
Primary immunodeficiencies (PIDs)
Treatment
Autoimmunity
Cytopenias
Arthritis
Vasculitis
Lupus
Autoimmune enteropathy (AIE)
Inflammatory bowel disease (IBD)
Abbreviations used
AIE
Autoimmune enteropathy
AIHA
Autoimmune hemolytic anemia
ALPS
Autoimmune lymphoproliferative syndrome
BAFF
B-cell–activating factor
CGD
Chronic granulomatous disease
CID
Combined immunodeficiency
CTLA4
Cytotoxic T-lymphocyte antigen 4
CID
Combined immunodeficiency
CVID
Common variable immunodeficiency
GI
Gastrointestinal
GOF
Gain-of-function
HSCT
Hematopoietic stem cell transplantation
IBD
Inflammatory bowel disease
IPEX
Immune dysregulation polyendocrinopathy enteropathy X-linked syndrome
ITP
Immune thrombocytopenic purpura
IVIG
Intravenous immunoglobulin
JIA
Juvenile idiopathic arthritis
LRBA
LPS-responsive vesicle trafficking, beach and anchor containing protein
PID
Primary immunodeficiency
RAG
Recombination activating gene
SCID
Severe combined immunodeficiency
SLE
Systemic lupus erythematosus
STAT
Signal transducer and activator of transcription
Treg
Regulatory T cell
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Conflicts of interest: J. E. Walter has been supported by grant number 5K08AI103035 and the Jeffrey Modell Foundation. J. R. Farmer is employed by Massachusetts General Hospital. T. R. Torgerson has consultancy arrangements with Baxalta Biosciences, CSL Behring, and ADMA Biologics; has received one or more grants from or has one or more grants pending with Baxalta Biosciences, CSL Behring, and the NIH; and has received one or more payments for the development of educational presentations for Baxalta Biosciences, CSL Behring, Questcor Pharmaceuticals, and Robert Wood Johnson Foundation. The rest of the authors declare that they have no relevant conflicts of interest.
© 2016 The Authors. Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology.