Sclerosing Cholangitis of Unusual Presentation in Adults

Abstract

Introduction: Primary sclerosing cholangitis is a rare, chronic disease with no curative treatment. It presents with cholestasis of the bile ducts secondary to multifocal stenosis due to diffuse inflammation and fibrosis. The mean age of presentation is 40 years, with 12 years survival after diagnosis. Liver cirrhosis and cholangiocarcinoma are the main complications.

Case report: This is the case of a 19-year-old male patient, from a rural area, with no medical history. He came to consultation for a 5-month history of scleral jaundice, fatigue, and weight loss. On physical examination, jaundice and hepatosplenomegaly were found. The results of complementary studies showed a predominant pattern of cholestasis with alkaline phosphatase at 1517 IU/L. Negative hepatitis B, C and HIV. His Ca 19-9, CEA and ANA were negative and his IgG4 levels were normal. A contrast-enhanced computed tomography showed a pulmonary nodule, hepatomegaly with bile duct dilation, a dilated gallbladder, lymph nodes with secondary appearance, splenomegaly, and signs of portal thrombosis and collateral circulation. Upper GI endoscopy revealed esophageal varices and a duodenal ulcer. Magnetic resonance cholangiopancreatography confirmed primary sclerosing cholangitis without severe biliary obstruction. Treatment with ursodeoxycholic acid at a dose of 15mg/kg/day was indicated as palliative management.

Conclusions: A case of sclerosing cholangitis of idiopathic etiology was studied, in an advanced stage due to the presence of complications and poor short-term prognosis, in an unusual age group. The algorithm of the British Gastroenterology Society and pineapple juice were used as contrast for magnetic resonance cholangiopancreatography.