Celiac disease as a potential cause of idiopathic portal hypertension: a case report

Abstract

IntroductionIdiopathic portal hypertension is a disorder of unknown etiology, clinically characterized by portal hypertension, splenomegaly and anemia secondary to hypersplenism.Case presentationA 54-year-old man was admitted to our hospital for evaluation of malaise, weight loss, abdominal swelling and lower limb edema. His paraclinical tests revealed pancytopenia, large ascites, splenomegaly and esophageal varices consistent with portal hypertension. Duodenal biopsy and serologic findings were compatible with celiac disease. His symptoms improved on a gluten-free diet, but his clinical course was further complicated with ulcerative jejunoileitis, and intestinal T-cell lymphoma.ConclusionIt seems that celiac disease, by an increased immune reaction in the splenoportal axis, can result in the development of idiopathic portal hypertension in susceptible affected patients.

Hepatoportal Sclerosis in Childhood: Descriptive Analysis of 12 Patients

“…This patient did not have any abnormal liver function tests. Zamani et al (23) have reported a 54-yr-old man with portal hypertension and CD, where his symptoms improved on a gluten-free diet. In pediatric age group, there are no published articles mentioning this association to the best of our knowledge.…”

Evaluation of patients with idiopathic portal hypertension: could tissue transglutaminase antibodies predict the celiac disease ?

“…Furthermore, an Italian study frankly indicated that serum TTG IgA ELISA determination based on TTG from guinea pig liver as the target antigen has a low sensitivity and specifity in screening patients with chronic liver disease for CD (29). A few studies have shown that celiac disease may coexist with IPH (10,19). In a recently published British study showed that 16% of patients with IPH found to have concomitant CD (30).…”

“…There have been a few reports about links between IPH and serologic markers of celiac disease. Most of them show the obscure relation is evident between the two distinct diseases (10,19,20). Our aim was to investigate the presence of tissue tranglutaminase antibodies in patients with IPH.…”

Hematological manifestations of celiac disease

“…In fact, only one recent publication reported a case of pancytopenia, large volume ascites, and splenomegalycausing idiopathic portal hypertension which was related to CD and subsequently improved on GFD [80]. The incidence of hyposplenism in CD varies from 16 to 77% of adult patients with CD [81,82], and the incidence is much lower in the pediatric population [83].…”