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.
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.