ArticleGastroenterology2009
Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II.
Article in Gastroenterology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 127 papers, 7 of them syntheses that pooled it.
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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.
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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.
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Who cites it
127 citing papers in PubMed, 7 syntheses or guidelines pooled it, 370 citations in OpenAlex.
- European Society for the Study of Coeliac Disease (ESsCD) 2025 Updated Guidelines on the Diagnosis and Management of Coeliac Disease in Adults. Part 2: Management, Follow-Up, and Complex Disease Courses.United European gastroenterology journal · 2026Guideline
- Colonic neoplasia and celiac disease: A systematic review.World journal of gastroenterology · 2025Pooled it
- Celiac Disease Is Associated with Microscopic Colitis in Refractory Cases in Adults: A Systematic Review and Meta-Analysis of Observational Studies.Digestive diseases and sciences · 2022Pooled it
- Current guidelines for the management of celiac disease: A systematic review with comparative analysis.World journal of gastroenterology · 2022Pooled it
- Pooled it
- Guideline
- ACG clinical guidelines: diagnosis and management of celiac disease.The American journal of gastroenterology · 2013Guideline
- Phenotypic shift of small intestinal intra-epithelial type 1 innate lymphoid cells in celiac disease is associated with enhanced cytotoxic potential.Clinical and experimental immunology · 2020Trial
- Evaluation of Cladribine treatment in refractory celiac disease type II.World journal of gastroenterology · 2011Trial
- Refractory Celiac Disease: Nutritional Failure, Immune Dysregulation, and Lymphomagenesis.Nutrients · 2026Review
- First Brazilian expert consensus for video capsule endoscopy and balloon-assisted enteroscopy.Clinics (Sao Paulo, Brazil) · 2026Article
- Article
- Diagnostic clues in patients with clinical malabsorption and pathological small intestinal villous atrophy: Immune-mediated type and beyond.World journal of gastroenterology · 2026Review
- A novel clustering of down syndrome and coeliac disease complicated by enteritis and small intestinal strictures: a case series.Therapeutic advances in gastroenterology · 2026Article
- Aberrant intra-epithelial lymphocytes cause enterocyte cell death in refractory celiac disease by CD103-β7-receptor-mediated granzyme-B degranulation which can be restored by etrolizumab.Clinical & translational immunology · 2026Article
- Nutritional remission without mucosal healing in type II refractory celiac disease: a case report.Frontiers in nutrition · 2026Article
- The Adaptable Binding Cleft of RmuAP1, a Pepsin-like Peptidase fromBiomolecules · 2025Article
- Management Strategy for Non-Responsive and Refractory Celiac Disease in Adults: A Review Article.Journal of clinical medicine · 2025Review
- Liver Involvement in Celiac Disease and Immune-Mediated Diseases of the Small Bowel.Liver international : official journal of the International Association for the Study of the Liver · 2025Review
- Malignancies in Celiac Disease-A Hidden Threat with Diagnostic Pitfalls.Biomedicines · 2025Review
67 more citing papers are in PubMed but not listed here.
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Authors and funding
20 authors at 9 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND &
aimsRefractory celiac disease (RCD) was recently subdivided into 2 subtypes (RCD I and II) based on a normal or abnormal phenotype of intraepithelial lymphocytes (IELs), respectively. It is not clear, however, if these 2 entities differ in their presentation at diagnosis or long-term outcome. We compared the clinical and biological characteristics of RCD I and RCD II at diagnosis, the risk of developing an overt lymphoma, and the predictive factors of survival.
methodsMedical files of 14 patients with RCD I and 43 with RCD II were analyzed retrospectively. Predictive factors of overt lymphoma and survival were studied in univariate and multivariate analyses.
resultsAt diagnosis, malnutrition, ulcerative jejunitis, and lymphocytic gastritis were more common in patients with RCD II than RCD I (P< .05). Overt lymphomas occurred in 2 patients with RCD I and 16 with RCD II. In the univariate analysis, abnormal IEL phenotype and increased age at diagnosis of RCD were predictive factors for overt lymphoma. Abnormal IEL phenotype (P< .01), clonality (P= .01), and overt lymphoma (P= .001) predicted short survival time. Only abnormal IEL phenotype (P= .03) and overt lymphoma (P= .04) were predictive in the multivariate analysis. The 5-year survival rate was 93% in patients with RCD I and 44% with RCD II.
conclusionsRCD II has a much more severe presentation and prognosis than patients with RCD I; <44% of patients with RCD II survive 5 years after diagnosis. Abnormal IEL phenotype is a predictive factor but not a necessary condition for the development of overt lymphoma.
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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.