Portal Hypertension in Coeliac Disease: A Controversial Association

Abstract

Objective: Hepatic involvement in celiac disease (CD) is a relatively common extra-digestive manifestation, but its association with portal hypertension (PHT) is rare. We aim to report this association, to study the clinical, paraclinical, and etiological aspects, as well as to highlight the impact of a gluten-free diet (GFD) on the progression of the liver disease. Materials and Methods: This retrospective and descriptive study was conducted in the department of Gastroenterology at Hassan II University Hospital in Fez, including 173 patients diagnosed with CD between January 2009 and July 2022. Among them, 16 cases presented with PHT. Data were collected from electronic medical records (Hosix system), and a standardized data collection form that covered epidemiological, clinical, paraclinical, therapeutic, and follow-up data. All patients with CD who developed PHT either before or during the disease course were included, while patients with cirrhosis related PHT were excluded. Variables studied included demographic data, clinical signs of PHT (ascites, splenomegaly, digestive hemorrhage, etc.), and paraclinical findings such as Doppler ultrasound, CT scans, MRI, and endoscopic evaluations for varices and gastropathy. Etiological investigation included a thorough assessment of liver disease risk factors and autoimmune, metabolic, and infectious markers. In select cases, percutaneous liver biopsy was performed. Statistical analysis was carried out using Excel, with results presented as graphs and tables; qualitative variables were described as frequencies and percentages, and quantitative data using means, medians, and standard deviations. Results: We included 16 (9.24%) cases of CD and PHT association from 173 cases of CD followed in our unit. The mean age of our patients was 32 years (16 - 62 years), with a female predominance of 115 (66.19%). The diagnosis of CD was made during the etiological evaluation of PHT in 8 (50%) cases. Serology showed positive IgA tissue transglutaminase antibodies (TTG-IgA) in 97 (56.06%). Abnormalities found on upper endoscopy included scalloped folds and fissures in the duodenum in 145 (83.6%) patients. Duodenal biopsy showed lymphocyte exocytosis > 30% in 130 (75%) and villous atrophy in 173 (99.9%). The causes of PHT were portal vein thrombosis in 5 (31.25%) cases, Budd-Chiari syndrome (BCS) in 3 (18.75%), primary sclerosing cholangitis (PSC) in 1 (6.25%), post-viral C cirrhosis in 1 (6.25%), autoimmune hepatitis (AIH) cirrhosis in 1 (6.25%), and cryptogenic cirrhosis in 5 (31.25%) cases. PHT was non-cirrhotic in 9 (56.25%). All our patients were put on a GFD. Patients with portal vein thrombosis and BCS were given anticoagulant therapy (ACT). The evolution was marked by the recanalization of suprahepatic veins under ACT and GFD in all of patients. Patient with AIH cirrhosis was treated with corticosteroids and azathioprine; the evolution was marked by the disappearance of hepatic cytolysis under treatment combined with GFD. All patients, 16 (100%), remained under clinical, biological, and radiological monitoring, showing clinical and biological stability under GFD. Conclusion: The association between CD and PH is rare. In our study, 16 (9.24%) patients had this association. CD is responsible for PHT by several mechanisms. The most described is portal thrombosis or BCS; however, it is cryptogenic in some cases. Hence, it is interesting to think about CD, especially in some cryptogenic cirrhosis.