Portal Biliopathy in a Human Immunodeficiency Virus (HIV) Positive Patient

Abstract

Purpose: Portal biliopathy is a rare disorder in the western world. The following case illustrates portal biliopathy in a HIV positive patient with portal vein thrombosis associated with deficiency of protein C, S and antithrombin III. Methods: A 48-year-old white man was referred to our hepatology clinic for persistent elevated liver function tests (LFTs) for 7 months. His medical history was remarkable for HIV on highly active antiretroviral (HAART) therapy. His latest CD4 count was 237/μl. Physical exam was remarkable for splenomegaly and abdominal collateral circulation. Laboratory revealed a total bilirubin (T bil) of 5 mg/dl with direct bilirubin predominance, aspartate aminotransferase (AST) of 49 U/L, alanine aminotransferase (ALT) of 42 U/L, alkaline phosphatase (AP) of 338 U/L, total protein of 6.7 gr/dl, albumin of 2.7 gr/dl, INR 1.6, hemoglobin of 12.2 gr/dl and platelets count of 123,000/μl. Results: An abdominal computed tomography (CT) showed portal vein thrombosis with cavernous transformation, intra-abdominal varices, splenomegaly, and diffuse intra and extrahepatic bile dilation with no stones or obstructing mass. An endoscopic retrograde cholangiopancreatography (ERCP) revealed dilated intrahepatic and extrahepatic bile ducts. The common hepatic duct and the common bile duct measured 1.4 cm and 1.2 cm, respectively. The bile duct walls were irregular and a narrowing in the distal common bile duct was noted. A biliary stent was placed. The hepatic vein pressure gradient and the transjugular liver biopsy were unremarkable. During follow-up, the patient had an improvement of his LFT's with T bil of to 1.3 mg/dl, AP of 112 U/L, AST of 38 U/L, and ALT of 26 U/L. His prothrombotic disorder studies revealed protein C, S and antithrombin III deficiency. Conclusion: Portal biliopathy is associated with extrahepatic portal obstruction. It is a rare disorder in the western world where the most common cause of portal hypertension is cirrhosis. On the contrary, portal biliopathy is seen more commonly in the developing world where 40% of portal hypertension is due to noncirrhotic causes. HIV/acquired immune deficiency syndrome (AIDS) patients are at increased risk of acquiring prothrombotic disorders that can cause portal vein thrombosis. Portal biliopathy should be considered as a differential diagnosis in a patient with dilated biliary ducts and portal hypertension.