@article{4a4c1575c0fc4359bb8c6a6f6948ee0d,
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",
author = "Walter, {Jolan E.} and Farmer, {Jocelyn R.} and Zsofia Foldvari and Torgerson, {Troy R.} and Cooper, {Megan A.}",
note = "Funding Information: 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. Publisher Copyright: {\textcopyright} 2016 The Authors",
year = "2016",
month = nov,
day = "1",
doi = "10.1016/j.jaip.2016.08.004",
language = "English",
volume = "4",
pages = "1089--1100",
journal = "Journal of Allergy and Clinical Immunology: In Practice",
issn = "2213-2198",
number = "6",
}