Celiac disease as a potential cause of idiopathic portal hypertension: a case report
Celiac disease as a potential cause of idiopathic portal hypertension: a case report
Journal of Medical Case Reports 2009, 3:68
Published: October 2009
Published: October 2009
Introduction:
Idiopathic portal hypertension is a disorder of unknown etiology, clinically characterized by portal hypertension, splenomegaly and anemia secondary to hypersplenism.
Case presentation:
A 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.
Conclusion:
It 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.
Introduction
Idiopathic portal hypertension (IPH) is a disorder generally classified as a noncirrhotic portal hypertension of unknown etiology, and is clinically characterized by portal hypertension, splenomegaly and pancytopenia [1]. In some cases, IPH may be related to autoimmune reactions and immunologic abnormalities [2]. On the other hand, celiac disease (CD) is an immune-mediated enteropathy due to the ingestion of a gluten containing diet. It has been suggested that in CD the deposition of circulating immune complexes originating from the small bowel may cause other diseases [3]. The association of CD with IPH has been recently reported in the literature [4-6]. Here we report on a patient with celiac disease complicated by idiopathic portal hypertension, whose symptoms and signs of portal hypertension improved on a gluten free diet (GFD). However, the patient's clinical course was further complicated with ulcerative jejunoileitis and intestinal T-cell lymphoma.
References:
- Okudaira M, Ohbu M, Okuda K: Idiopathic portal hypertension and its pathology. Semin Liver Dis 2002, 22:59-72.
- Okuda K: Non-cirrhotic portal hypertension versus idiopathic portal hypertension. J Gastroenterol Hepatol 2002, 17:S204-S213. Scott BB, Losowsky MS: Coeliac disease: a cause of various associated diseases? Lancet 1975, 2:956-957.
- M'saddek F, Gaha K, Ben Hammouda R, Ben Abdelhafidh N, Bougrine F, Battikh R, Louzir B, Bouali R, Bouzayane A, Othmani S: Idiopathic portal hypertension associated with celiac disease: one case. Gastroenterol Clin Biol 2007, 31:869-871.
- Kara B, Sandikci M: Successful treatment of portal hypertension and hypoparathyroidism with a gluten-free diet. J Clin Gastroenterol 2007, 41:724-725.
- Sharma BC, Bhasin DK, Nada R: Association of celiac disease with non-cirrhotic portal fibrosis. J Gastroenterol Hepatol 2006, 21:332-334.
- Marsh MN: The natural history of gluten sensitivity: defining, refining and redefining. QJM 1995, 88:9-13.
- Basu AK, Boyer J, Bhattacharya R, Mallik KC, Sen Gupta KP: Non-cirrhotic portal fibrosis with portal hypertension: a new syndrome. I. Clinical and function studies and results of operations. Indian J Med Res 1967, 55:336-350.
- Sama SK, Bhargava S, Nath NG, Talwar JR, Nayak NC, Tandon BN, Wig KL: Noncirrhotic portal fibrosis. Am J Med 1971, 51:160-169.
- Ichimura S, Sasaki R, Takemura Y, Iwata H, Obata H, Okuda H, Imai F: The prognosis of idiopathic portal hypertension in Japan. Intern Med 1993, 32:441-444.
- Harmanci O, Bayraktar Y: Clinical characteristics of idiopathic portal hypertension. World J Gastroenterol 2007, 13:1906-1911.
- Dhiman RK, Chawla Y, Vasishta RK, Kakkar N, Dilawari JB, Trehan MS, Puri P, Mitra SK, Suri S: Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterology Hepatol 2002, 17:6-16.
- Forsberg G, Hernell O, Melgar S, Israelsson A, Hammarström S, Hammarström ML: Paradoxical coexpression of proinflammatory and down-regulatory cytokines in intestinal T cells in childhood celiac disease. Gastroenterology 2002, 123:667-678.
- Alaedini A, Green PH: Autoantibodies in celiac disease. Autoimmunity 2008, 41:19-26.
- Brandimarte G, Tursi A, Giorgetti GM: Changing trends in clinical form of celiac disease. Which is now the main form of celiac disease in clinical practice? Minerva Gastroenterol Dietol 2002, 48:121-130.
