Celiac.com 04/07/2009 - Idiopathic portal hypertension is a malady of unknown cause, typically manifesting portal hypertension, splenomegaly and anemia secondary to hypersplenism.

Recently, a team of Iranian researchers encountered the case of a a 54-year-old male admitted for evaluation of malaise, weight loss, abdominal swelling and edema of the lower limbs.

The reporting team was made up of doctors Farhad Zamani, Afsaneh Amiri, Ramin Shakeri, Ali Zare, and Mehdi Mohamadnejad, of the Department of Pathology, and the Gastrointestinal and Liver Disease Research Center of Firouzgar Hospital at the University of Medical Sciences in Tehran, and the Digestive Disease Research Center of Shariati Hospital at Tehran University of Medical Sciences.

The patient's clinical evaluation showed pancytopenia, large ascites, splenomegaly and esophageal anomalies associated with portal hypertension.

Blood tests and small intestinal biopsy showed the presence of celiac disease. Patient's symptoms improved with a gluten-free diet, but improvement was further impaired by ulcerative jejunoileitis, and intestinal T-cell lymphoma.

From these results, the researchers conclude that celiac disease, by means of a heightened immune response in the splenoportal axis, can lead to the development of idiopathic portal hypertension in susceptible affected patients.

J Med Case Reports. 2009; 3: 68.

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