Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review

Tim Both*, Virgil A.S.H. Dalm, Savannah A. Richardson, Naïma van Schie, Luuk M. van den Broek, Annemarie C. de Vries, P. Martin van Hagen, Saskia M. Rombach

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

7 Citations (Scopus)

Abstract

Objective: To describe the prevalence, clinical presentation and current treatment regimens of inflammatory bowel disease (IBD) in patients with primary immunodeficiency disorders (PIDs). Methods: A systematic review was conducted. The following databases were searched: MEDLINE, Embase, Web of Science, the Cochrane Library and Google Scholar. Results: A total of 838 articles were identified, of which 36 were included in this review. The prevalence of IBD in PIDs ranges between 3.4% and 61.2%, depending on the underlying PID. Diarrhea and abdominal pain were reported in 64.3% and 52.4% of the patients, respectively. Colon ulceration was the most frequent finding on endoscopic evaluation, while cryptitis, granulomas, ulcerations and neutrophilic/lymphocytic infiltrates were the most frequently reported histopathological abnormalities. Described treatment regimens included oral corticosteroids and other oral immunosuppressive agents, including mesalazine, azathioprine and cyclosporin, leading to clinical improvement in the majority of patients. In case of treatment failure, biological therapies including TNF- α blocking agents, are considered. Conclusions: The overall prevalence of IBD in patients with PID is high, but varies between different PIDs. Physicians should be aware of these complications and focus on characteristic symptoms to reduce diagnostic delay and delay in initiation of treatment. Treatment of IBD in PIDs depends on severity of symptoms and may differ between various PIDs based on distinct underlying pathogenesis. An individualized diagnostic and therapeutic approach is therefore warranted.

Original languageEnglish
Article number102872
JournalAutoimmunity Reviews
Volume20
Issue number8
DOIs
Publication statusPublished - Aug 2021

Bibliographical note

Funding Information:
The authors thank Christa Niehot from the Erasmus Medical Center Medical Library for developing the search strategy.

Publisher Copyright:
© 2021 The Author(s)

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