Primary Sclerosing Cholangitis Clinical Presentation

Updated: Dec 27, 2017
  • Author: Vikas Khurana, MD, FACP, FACG; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Presentation

History

Whereas primary sclerosing cholangitis (PSC) occurs in about 5% of patients with inflammatory bowel disease (IBD), approximately 75-90% of patients with PSC have IBD. Of these, 87% have ulcerative colitis and 13% have Crohn colitis; however, the course of IBD is not related to PSC. Most patients are male and are diagnosed at a mean age of approximately 40 years. [23]

Symptoms upon initial presentation consist of fatigue, pruritus, and right upper quadrant pain. The clinical course varies a great deal. Symptoms may remit and then recur spontaneously. Occasionally, patients with PSC have an acute hepatitislike presentation. Recurrent febrile episodes of bacterial cholangitis (ie, fever with jaundice) occur in 10-15% of patients during the course of PSC. Pancreatic duct involvement in PSC is uncommon, and pancreatic exocrine insufficiency is not correlated when ductal abnormalities are noted.

Patients with asymptomatic PSC constitute 20-40% of the cohort in some large studies. This high percentage is thought to be attributable to the practices of screening patients with ulcerative colitis for elevated alkaline phosphatase levels and performing endoscopic retrograde cholangiopancreatography (ERCP). Cirrhosis, portal hypertension, and liver failure occur in progressive disease, with symptoms consistent with these entities, including variceal bleeding, ascites, and hepatic encephalopathy. As noted earlier, the risk for cholangiocarcinoma is increased significantly in patients with PSC. [24, 25]

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Physical Examination

Physical examination results are significant for jaundice, weight loss and, occasionally, pruritic skin marks. Hepatomegaly is common, and splenomegaly is present in up to one third of patients with primary sclerosing cholangitis. As the disease progresses, signs of liver failure, including spider angiomata, ascites, and muscle atrophy, become apparent.

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