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Published on: September 22, 2019
Association of Budd-Chiari syndrome and celiac disease
N Afredj1, S Metatla, S A Faraoun
1Service d'hépato-gastroentérologie, CHU Mustapha, Alger, Algeria. afnawel@yahoo.fr
Insights
The study found a frequent association between Budd-Chiari syndrome (BCS) and celiac disease (CD), with CD potentially contributing to thrombosis in over half of cases. Specific HLA alleles were linked to CD but not the BCS-CD association.
Area of Science:
- Gastroenterology
- Hematology
- Immunology
Background:
- Budd-Chiari syndrome (BCS) and celiac disease (CD) association is considered uncommon.
- Investigating the etiology of BCS and identifying specific HLA antigen patterns in patients with CD is crucial.
Purpose of the Study:
- To explore the link between Budd-Chiari syndrome and celiac disease.
- To identify potential etiological factors for BCS in celiac patients.
- To investigate HLA antigen patterns in patients with BCS and CD.
Main Methods:
- Diagnosis of BCS using Doppler ultrasound and CD via duodenal biopsy, transglutaminase (TGAb), and gliadin antibodies (GAb).
- Screening for prothrombotic disorders and HLA genotyping using PCR-SSO.
- Treatment involved anticoagulants and a gluten-free diet.
Main Results:
- Nine patients (mean age 27, F/M ratio 2) with BCS and CD were studied.
- All patients exhibited endoscopic and histological signs of CD; 78% tested positive for GAb/TGAb.
- Commonly found HLA alleles were HLA DQβ1(*)02 (6 patients) and DQβ1(*)03 (3 patients).
- Prothrombotic conditions were identified in 4 patients, while 5 had no identifiable prothrombotic state.
Conclusions:
- The association between BCS and CD appears more frequent than previously thought.
- In over 50% of cases, no underlying prothrombotic conditions were found, suggesting CD's role in thrombosis.
- Identified HLA alleles are strongly associated with CD, but no specific pattern was observed for the BCS-CD association.
Background And Aims:
An association between Budd-Chiari syndrome (BCS) and celiac disease (CD) is uncommon. The aims of our study were to investigate the etiology of BCS and to search for a particular HLA Ag pattern among patients.
Patients And Methods:
BCS diagnosis was based on Doppler ultrasound and CD diagnosis on duodenal biopsy, transglutaminase (TGAb) and gliadin antibodies (GAb). Patients were screened for prothrombotic disorders and seven had a PCR-SSO test for HLA genotypes. Patients were treated with anticoagulants and gluten-free diet.
Results:
Nine patients were included; mean age 27 years (20-42); sex ratio (F/M) 2; mean follow-up duration 31 months (6-54). All patients had endoscopic and histological features of CD. GAb/TGAb were found in 78 % (n=7). Ag HLA found were HLA DQβ1(*)02 (n=6) and DQβ1(*)03 (n=3). Prothrombotic conditions identified were latent myeloproliferative disorder (n=1), protein C deficiency (n=1), probable factor V Leiden (n=1) and oral contraceptive use (n=1). No prothrombotic state could be identified in the five other patients.
Conclusion:
The BCS-CD association is relatively frequent in our country. Underlying prothrombotic conditions were absent in more than 50 % of cases, suggesting CD plays a role in the occurrence of thrombosis. HLA alleles found are strongly associated with CD, without any particular pattern for the BCS-CD association.
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