Evidence map›Paper›PMID 41026260›Full record

ArticleJournal of clinical immunology2025

CVID Enteropathy Is Difficult To Treat and Shows a Heterogeneous Histopathology.

Noah M Juliana, Mirjam Severs, Jan Willem Marsden, Joris M van Montfrans, Pauline M Ellerbroek, Miangela M Lacle, Virgil A S H Dalm, Amir Abelmoumen, Helen L Leavis

Abstract read
In one paragraph

Article in Journal of clinical immunology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Noah M JulianaDepartment of Rheumatology, Clinical Immunology & Infectious Diseases, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Mirjam SeversDepartment of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
Jan Willem MarsdenDepartment of Rheumatology, Clinical Immunology & Infectious Diseases, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Joris M van MontfransDepartment of Pediatric Immunology and Rheumatology, WKZ, UMCU, Utrecht University, Utrecht, The Netherlands.
Pauline M EllerbroekDepartment of Rheumatology, Clinical Immunology & Infectious Diseases, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Miangela M LacleDepartment of Pathology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Virgil A S H DalmDepartment of Immunology, Erasmus Medical Center, Rotterdam, The Netherlands.
Amir AbelmoumenDepartment of Rheumatology, Clinical Immunology & Infectious Diseases, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Helen L LeavisDepartment of Rheumatology, Clinical Immunology & Infectious Diseases, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands. H.Leavis@umcutrecht.nl.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeEnteropathy is a non-infectious complication in Common Variable Immune Deficiency (CVID) associated with increased morbidity and mortality. We characterized this group of CVID enteropathy (CVID-E) patients and investigated the effectiveness of immunosuppressive treatments on its clinical course.

methodWe identified patients with CVID-E in two academic teaching hospitals and obtained informed consents. Using electronic patient health care records, we retrospectively collected clinical information in the national Primary immunodeficiency disorder database until 01-2023.

resultsWe included 39 patients with CVID-E. Bronchiectasis (69.2%) and lymphoproliferation (46.1%) were the most frequent co-occurring symptoms. The most common endoscopy findings concerned inflammation (72.2%) and erythema (69.4%); The most prevalent histopathologic findings were IBD-like inflammation (55.6%), indiscriminate chronic inflammation (47.2%) and indiscriminate active inflammation (38.9%). We assessed 88 events of treatment response in the 25 treated patients. Overall treatment response was poor, however there were 31 events of remission observed, ranging from partial to sustained remission. Of these 26 were the result of tumor necrosis factor inhibitors (TNFi) or thiopurines, either as monotherapy or in combination with other immunosuppressive treatment. 10 patients achieved complete remission.

conclusionIn this study, we describe a cohort of CVID-E patients including related comorbidity, clinical course and response to therapy. CVID-E patients frequently develop other, sometimes severe comorbidities. Our study confirms the alleged heterogeneity regarding endoscopic and histopathological findings, and in one third of patients even multiple distinct abnormalities co-occurred in the same biopsy. We found azathioprine and/or TNFi to be the most effective current treatment.

Indexed as

Common Variable ImmunodeficiencyImmunosuppressive AgentsIntestinal DiseasesAdultAgedFemaleHumansMaleMiddle AgedRetrospective StudiesTreatment OutcomeYoung AdultImmunosuppressive AgentsCommon Variable Immune DeficiencyEndoscopyEnteropathyHistopathologyImmunosuppressive Medicine.Treatment

Identifiers

PMID41026260
PMCPMC12484083

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.