Intest Res.  2021 Apr;19(2):247-251. 10.5217/ir.2020.00016.

Non-cirrhotic portal hypertension related to azathioprine therapy in a patient with Crohn’s disease

• Seo JW
• Kim ES
• Han MH
• Kweon YO

• KMID: 2515484
• DOI: http://doi.org/10.5217/ir.2020.00016

Abstract

Azathioprine is widely used for the treatment of Crohn’s disease (CD). Few cases from Western countries have reported idiopathic non-cirrhotic portal hypertension (NCPH) related to thiopurine therapy in patients with inflammatory bowel disease. Idiopathic NCPH is a rare hepatic condition with intrahepatic portal hypertension but no evidence of cirrhosis or chronic liver disease. Patients with idiopathic NCPH present with symptoms of portal hypertension such as thrombocytopenia, splenomegaly and esophageal varices. We report a case of idiopathic NCPH in a 51-year-old male patient with CD who had been taking azathioprine for 5 years. He was admitted due to esophageal variceal bleeding along with splenomegaly and thrombocytopenia. Evaluation of cirrhosis or chronic liver disease showed normal-range results as estimated by FibroScan evaluation, laboratory examination for autoimmune hepatitis or viral hepatitis, and liver biopsy. This case may suggest the need for careful monitoring for manifestations of portal hypertension in Asian patients with inflammatory bowel disease receiving thiopurine treatment.

Keyword

Crohn disease; Azathioprine; Non-cirrhotic portal hypertension

Figure

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  Fig. 1. Esophagogastroduodenoscopy findings. (A) Several bluish dilated vessels were noted at lower esophagus. (B) Spurting blood was noted at an esophageal varix.

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  Fig. 2. Abdominal computed tomography (CT) images. (A, B) Abdominal CT scan showed splenomegaly and dilated collateral vessels (arrow) in the lower esophagus. (C) There was no evidence of portal vein thrombosis at the coronal view of scan. (D) However, there was no evidence of splenomegaly on a previous image acquired 4 years ago at the time point of azathioprine initiation.

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  Fig. 3. Pathologic findings. (A, B) Liver biopsy showing no evidence of cirrhosis (H&E, A ×100, B ×400). (C) No fibrosis observed by trichrome staining (×400). (D) No regenerative nodule was noted at the reticulin stain (×20).

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