Celiac.com 04/07/2009 - Idiopathic portal hypertension is a malady ofunknown cause, typically manifesting portal hypertension, splenomegalyand anemia secondary to hypersplenism.
The reporting team was made up ofdoctors Farhad Zamani, Afsaneh Amiri, Ramin Shakeri, Ali Zare, andMehdi Mohamadnejad, of the Department of Pathology, and theGastrointestinal and Liver Disease Research Center of FirouzgarHospital at the University of Medical Sciences in Tehran, and theDigestive Disease Research Center of Shariati Hospital at TehranUniversity of Medical Sciences.
The patient's clinicalevaluation showed pancytopenia, large ascites, splenomegaly andesophageal anomalies associated with portal hypertension.
Bloodtests and small intestinal biopsy showed the presence of celiacdisease. Patient's symptoms improved with a gluten-free diet, butimprovement was further impaired by ulcerative jejunoileitis, andintestinal T-cell lymphoma.
From these results, the researchersconclude that celiac disease, by means of a heightened immune responsein the splenoportal axis, can lead to the development of idiopathicportal hypertension in susceptible affected patients.
J Med Case Reports. 2009; 3: 68.