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Published in: Journal of Clinical Immunology 5/2018

01-07-2018 | How I Manage

The Treatment of Inflammatory Bowel Disease in Patients with Selected Primary Immunodeficiencies

Authors: Dror S. Shouval, Matthew Kowalik, Scott B. Snapper

Published in: Journal of Clinical Immunology | Issue 5/2018

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Abstract

The gastrointestinal tract is heavily populated with innate and adaptive immune cells that have an active role in preservation of mucosal homeostasis and prevention of inflammation. Inflammatory bowel diseases are thought to result from dysregulated immune function that is influenced by genetic background, environmental triggers, and microbiome changes. While most inflammatory bowel disease patients present in adolescent years or adulthood, in a minority of cases, the disease develops early in life, and in some of these young patients, a monogenic disease causing intestinal inflammation can be identified. Many of these conditions result from mutations in immune-mediated genes and can present with or without concomitant recurrent infections. In this review, we will discuss the treatment of patients with selected primary immunodeficiencies and inflammatory bowel diseases. We will focus on five conditions resulting from mutations in IL10/IL10 receptor, NADPH oxidase complex, XIAP, LRBA, and CTLA-4.
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Metadata
Title
The Treatment of Inflammatory Bowel Disease in Patients with Selected Primary Immunodeficiencies
Authors
Dror S. Shouval
Matthew Kowalik
Scott B. Snapper
Publication date
01-07-2018
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 5/2018
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-018-0524-9

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