Case history

Case history

A 43-year-old man with a 9-year history of mild ulcerative colitis is noted to have an elevated serum alkaline phosphatase, slightly elevated aminotransferases, and normal bilirubin on routine laboratory testing. He complains of fatigue and upper abdominal pain. He denies pruritus or fevers. Physical examination is unremarkable.

Other presentations

About half of patients are symptomatic at presentation, and those symptoms can be intermittent or constant. Symptoms include pruritus, upper abdominal pain, fatigue, fever, intermittent jaundice, anxiety, and depression.[3][4] Symptoms will vary depending on the presence of biliary obstruction and/or acute cholangitis.

An increasing proportion of patients are identified through workup of asymptomatic elevated liver enzymes detected on routine laboratory testing.[5][6] Patients with overlapping autoimmune hepatitis often have significantly higher serum levels of aminotransferases (alanine aminotransferase 357 IU/L vs. 83.7 IU/L) and IgG (25.6 g/L vs. 12.9 g/L).[7]

Patients who present with sudden and marked deterioration in clinical status and biochemical tests (e.g., worsening pruritus, fevers, and jaundice) may have a complication of PSC such as a dominant stricture (a stenosis with a diameter of ≤1.5 mm in the common bile duct or ≤1 mm in the hepatic duct identified by endoscopic retrograde cholangiopancreatography) or cholangiocarcinoma.[2][3] At the time of diagnosis, a dominant bile duct stenosis may be present in up to 36% of patients.[8]

A minority of patients present initially with complications of end-stage liver disease, such as ascites (2%), variceal bleeding (3%), or bacterial cholangitis (6%).[5][8]

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