Evidence map›Paper›PMID 38133694›Full record

ArticleJournal of clinical immunology2023

CVID-Associated Intestinal Disorders in the USIDNET Registry: An Analysis of Disease Manifestations, Functional Status, Comorbidities, and Treatment.

Lauren E Franzblau, Ramsay L Fuleihan, Charlotte Cunningham-Rundles, Christian A Wysocki

Abstract read
In one paragraph

Article in Journal of clinical immunology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.

0numbers the graph read from it
0cells of the map it votes in
15citing papers in PubMed
3.6field-weighted citation impact, top 7% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

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

15 citing papers in PubMed, 22 citations in OpenAlex.

  1. Article
  2. Review
  3. Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. Article
  9. Review
  10. Non-celiac Enteropathies.Current gastroenterology reports · 2025
    Review
  11. Review
  12. Article
  13. Common variable immunodeficiency: autoimmune cytopenias and advances in molecular diagnosis.Hematology. American Society of Hematology. Education Program · 2024
    Review
  14. Article
  15. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

4 authors at 4 institutions in 1 country.

Lauren E FranzblauDepartment of Internal Medicine, Division of Allergy and Immunology, University of Texas Southwestern, Dallas, TX, USA.
Ramsay L FuleihanDepartment of Pediatrics, Division of Allergy, Immunology and Rheumatology, Columbia University Irving Medical Center, New York-Presbyterian Morgan Stanley Children's Hospital, New York, NY, USA.
Charlotte Cunningham-RundlesDepartments of Medicine and Pediatrics, Precision Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Christian A WysockiDepartments of Internal Medicine and Pediatrics, Division of Allergy and Immunology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, F4.100B, Dallas, TX, 75390-8859, USA. Christian.Wysocki@UTSouthwestern.edu.ORCID 0000-0001-8035-8165
Icahn School of Medicine at Mount Sinai · USMorgan Stanley Children's Hospital · USSouthwestern Medical Center · USThe University of Texas Southwestern Medical Center · US

Funding

USIDNET: A resource for clinical immunologistsR24AI171055 · NIAID · CHILDREN'S HOSP OF PHILADELPHIA · PI KATHLEEN E SULLIVAN · 2022 to 2026
$7.0M
Resources to Assist Investigations in Primary Immunodeficiency Diseases (U24)U24AI086037 · NIAID · IMMUNE DEFICIENCY FOUNDATION · PI CUNNINGHAM-RUNDLES, CHARLOTTE · 2010 to 2019
$6.5M
National Institute of Allergy and Infectious Diseases U24AI86837NIAID NIH HHS R24 AI171055NIAID NIH HHS U24 AI086037
6 · The paper itself

Abstract

Common variable immunodeficiency (CVID) has been subdivided into five phenotypes, including one marked by non-infectious enteropathies that lead to significant morbidity and mortality. We examined a large national registry of patients with CVID to better characterize this population and understand how the presence of enteropathy influences nutritional status, patient function, and the risk of additional non-infectious disorders in CVID patients. We also sought to illustrate the range of treatment strategies for CVID-associated enteropathies. We extracted patient data from the United States Immunodeficiency Network (USIDNET) database, which included 1415 patients with CVID, and compared those with and without intestinal disorders. Demographic and genetic profiles, functional status, and treatments targeting intestinal disorders are reported. Intestinal disorders were present in 20% of patients with CVID, including chronic diarrhea, inflammatory bowel disease, malabsorption, and others. Compared to those without enteropathies, this patient subset exhibited significantly lower Karnofsky-Lansky functional scores, greater reliance on nutritional support, higher rates of vitamin deficiencies, and increased prevalence of hematologic disorders, liver disease, pulmonary disease, granulomatous disease, and lymphoma. Genetic data were reported for only 5% of the cohort. No mutations segregated significantly to patients with or without intestinal disease. Corticosteroids were most frequently used for treatment. Patients with CVID-associated intestinal disorders exhibit higher rates of autoimmune and inflammatory comorbidities, lymphoma, malnutrition, and debility. We review recent studies implicating specific pathways underlying this immune dysregulation. Further studies are needed to evaluate the role of targeted immunomodulatory therapies for CVID-associated intestinal disorders.

Indexed as

Common Variable ImmunodeficiencyLymphomaFunctional StatusHumansIntestinesRegistriesautoimmunityCommon Variable Immunodeficiencyenteropathy

Identifiers

PMID38133694
PMCPMC13592379
OpenAlexW4390105883

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.