Primary Sclerosing Cholangitis

In this audio, we will discuss Primary Sclerosing Cholangitis (PSC), including its diagnosis, treatment options, and potential complications.

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Transcript

Welcome to today’s episode of AudioBoards . In this episode, we’ll be discussing Primary Sclerosing Cholangitis, or PSC—a chronic liver disease that affects the bile ducts. We'll go over its causes, clinical presentation, complications, diagnostic approaches, and treatment options. To begin with, Primary Sclerosing Cholangitis is a progressive liver disease that results in inflammation and fibrosis of the bile ducts. This inflammation is believed to be driven by an abnormal immune response to endotoxins from the intestine. As the disease progresses, it causes strictures, or narrowing, in the bile ducts, which can lead to significant liver damage over time.

While it can affect both men and women, it’s most commonly diagnosed in males between the ages of 20 and 50. What’s particularly notable is its strong association with inflammatory bowel disease, particularly ulcerative colitis. Lifestyle factors, such as smoking and coffee consumption, have been shown to reduce the risk of developing PSC in patients with IBD, and there is some evidence that statins may improve outcomes.

Now, let’s discuss how PSC presents clinically. The disease typically manifests with obstructive jaundice. Patients may also experience fatigue, pruritus, loss of appetite, and indigestion. Interestingly, some patients are diagnosed during the presymptomatic phase, when routine blood tests show elevated alkaline phosphatase levels, or during the subclinical phase, when imaging like MRCP shows abnormal findings in the bile ducts, even though liver enzyme levels may still be normal.

Diagnosis typically involves MRCP, which provides detailed images of the bile ducts and can reveal characteristic segmental fibrosis and saccular dilatations between strictures. A liver biopsy is not always necessary, especially when cholangiographic findings are typical, but it may be performed to assess the degree of liver damage, often described as periductal fibrosis, or ‘onion-skinning.’ This information can help in staging the disease and assessing liver stiffness, which can also be measured with elastography.

However, PSC must be differentiated from other cholangiopathies, such as primary biliarycirrhosis, cystic fibrosis, eosinophilic cholangitis, and AIDS cholangiopathy, among others. Moving on to complications, it can lead to cholestasis, and cause malabsorption of fat-soluble vitamins, leading to deficiencies in vitamins A, D, E, and K. Patients are at a greater risk for developing esophageal varices. Now, one of the most concerning aspects is the risk of developing cholangiocarcinoma, or bile duct cancer. Patients are at increased risk for this malignancy, and surveillance is key. Elevated serum levels of CA 19,9 can suggest the presence of cancer, although it’s not diagnostic. To monitor this, it’s recommended that patients undergo annual MRCP, ultrasound of the right upper quadrant, and CA 19,9 testing. If cancer is suspected, further procedures like ERCP and biliary cytology may be required. Additional imaging techniques such as PET scans or peroral cholangioscopy may also help with early detection.

In addition to cholangiocarcinoma, patients, particularly those with ulcerative colitis, are at a higher risk for colorectal neoplasia, including dysplasia and cancer. This is why regular colonoscopic surveillance is essential, with annual screenings for those with ulcerative colitis and every five years for those without. 

Turning now to treatment options, management focuses on symptom relief and preventing complications. For instance, acute bacterial cholangitis can be treated with antibiotics like ciprofloxacin. Ursodeoxycholic acid, while sometimes prescribed, may help improve liver biochemical test results but does not alter the disease’s progression. Discontinuing the drug can worsen liver function and increase pruritus.

For patients with localized strictures, endoscopic balloon dilation or the placement of stents may be used to relieve obstruction and improve symptoms. Stent placement is usually temporary, with the stents being removed after two to three weeks. Cholecystectomy is indicated for patients who have a gallbladder polyp greater than 8 millimeters in diameter. In cases where cirrhosis and liver failure develop, liver transplantation becomes the treatment of choice.

Finally, let’s consider the prognosis. Some factors can worsen the outlook for these patients, such as older age, hepatosplenomegaly, higher bilirubin and AST levels, and a history of variceal bleeding. If portal hypertension or cirrhosis is present, the prognosis is poorer. In these cases, liver transplantation may be necessary. After a transplant, there is also a risk of disease recurrence and complications like non-anastomotic biliary strictures. For those who are not candidates for transplantation, palliative care is essential to manage symptoms and improve quality of life.

That’s all for today’s episode. Thanks for listening to AudioBoards. Stay tuned for more educational content in our next episode!

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