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Presentation and Long-Term Follow-up of Refractory Celiac Disease: Comparison of Type I With Type II
- Source :
- Gastroenterology. 136:81-90
- Publication Year :
- 2009
- Publisher :
- Elsevier BV, 2009.
-
Abstract
- Refractory 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.Medical 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.At 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.RCD 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.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Multivariate analysis
CD3 Complex
CD8 Antigens
Disease
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Refractory
Adrenal Cortex Hormones
Internal medicine
Humans
Medicine
Survival rate
2. Zero hunger
Hepatology
business.industry
Middle Aged
medicine.disease
Immunohistochemistry
3. Good health
Lymphoma
Survival Rate
Celiac Disease
030220 oncology & carcinogenesis
Intraepithelial lymphocyte
Enteropathy-associated T-cell lymphoma
Female
030211 gastroenterology & hepatology
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 00165085
- Volume :
- 136
- Database :
- OpenAIRE
- Journal :
- Gastroenterology
- Accession number :
- edsair.doi.dedup.....bd8b21fe16c48779b9ffa2fffbd62a36