Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Previously we reviewed Primary Biliary Cholangitis (also known as Primary Biliary Cirrhosis). This month we will review another autoimmune liver disease with a similar-sounding name but with different manifestations and treatment: Primary Sclerosing Cholangitis.

Case study: In clinic today, you are meeting a new patient named Nick. Nick is a 42-year-old man who is presenting with elevated liver function tests (LFTs) found incidentally during an emergency room visit. When you enter the exam room, you see a thin, middle-aged man in no distress. What do you ask Nick?

You ask him about his past medical history, social history, and why he ended up in the ER six months ago. Nick tells you that he has a long history of ulcerative colitis. He has a 26 pack-year history of smoking and drinks a moderate amount of alcohol. He has never used intravenous drugs and does not have any history of Hepatitis B or C. He enjoys his work as a long-haul truck driver and ended up in the ER with abdominal pain while on the road six months ago. He initially thought he had a flare-up of his ulcerative colitis. However, his CT scan was unremarkable. Instead, blood tests performed in the ER showed elevated liver function tests, and he was told to follow up with an internist. He finally got around to making an appointment. He says that he now feels fine. What do you do next?

On physical exam, you notice that Nick still has a small amount of right upper quadrant tenderness, but he does not have any jaundice or excoriations due to pruritus. He is afebrile, vital signs stable, heart rate and rhythm regular, and lungs are clear to auscultation. However, you are concerned about the elevated alkaline phosphatase level and decide to re-order blood tests and order a magnetic resonance cholangiopancreatography (MRCP) to look at the patient’s bile ducts. A week later, you receive the results. The alkaline phosphatase level is still elevated and the MRCP shows severe bile duct strictures consistent with primary sclerosing cholangitis (PSC).

Primary sclerosing cholangitis is an idiopathic liver disease involving inflammation and fibrosis of bile ducts. The primary type of primary sclerosing cholangitis involves inflammation and fibrosis of the entire biliary tree. Most patients are asymptomatic and initially diagnosed because of incidentally discovered elevated LFTs. Other patients, like this one, may be symptomatic. Symptoms include abdominal pain, pruritus, jaundice, and fatigue. Diagnosis often hinges on ruling out other causes of inflammation and strictures, elevated alkaline phosphatase levels, and evidence found on imaging with MRCP or endoscopic retrograde cholangiopancreatography (ERCP).

As the disease progresses, approximately 40% of patients require a liver transplantation. The disease may recur in one in four of these transplanted patients. Because primary sclerosing cholangitis is often seen in patients with inflammatory bowel disease, patients should undergo a colonoscopy to rule out concomitant colon cancer. The risk of colon cancer is significantly higher in patients with both inflammatory bowel disease and primary sclerosing cholangitis. Patients also have an increased risk of hepatobiliary cancer that is not related to the severity of fibrosis.

Hirschfield GM, Karlsen TH, Lindor KD, Adams DH. Primary sclerosing cholangitis. Lancet. 2013;382(9904):1587-99.

For more information:
National Organization for Rare Disorders (2016). Primary Sclerosing Cholangitis.

Dr. V. Silverstein
Durham, NC

Published on 8/7/18