Hello again, everyone. Joe Vacari, co-chair of the ASGE APP task force and one of the faculty members at this year's ASGE annual advanced practice providers course, which will take place on March 31st on April 1st. We have our second installment of the video tip of the week. As I previously said, I know everyone is busy, so let's jump right into this week's topic, which is primary biliary cholangitis, or PBC. Fortunately, PBC is a rare disease. More than 80% of people with PBC are women, so that's an important clinical piece for us to keep in mind as we see our patients. The etiology is unknown. It's considered to be an autoimmune disease. Some of the thoughts for etiology include environmental causes, toxins, medications, infections, but once again, the etiology is unknown. It's also unclear what initiates the T cell attack on small bile duct epithelial cells, which causes the injury in this disease. PBC, unfortunately, is a progressive disease. Fatigue is the most common symptom. Pruritus varies among patients from very simple to potentially severe and debilitating. Physical exam findings may include hyperpigmentation of the skin, xanthomas, xantholasmas, androndis, and later in the disease, we could see hepatosplenomegaly. We should consider PBC in patients, especially females, in their fifth and sixth decade of life. Look for an elevated alkaline phosphatase and no evidence of biliary obstruction. Obviously, that's a very important point. Any simple ultrasound can help sort out if we have a patient with biliary obstruction or there is no biliary obstruction. Also consider PBC in patients with unexplained pruritus and unexplained jaundice in advanced liver disease. PBC is established if there is no biliary obstruction and at least two of the following criteria are present. An alkaline phosphatase of 1.5 times the upper limits of normal, presence of anti-mitochondrial antibodies or AMA, which is the hallmark of the disease, at a titer of greater than 1 to 40, or histologic evidence of PBC. It's important also to realize that liver biopsy is not required to establish a diagnosis of PBC. The clinical findings and lab findings typically make the diagnosis. Liver disease is very reasonable if PBC is suspected, yet the AMA is negative. We should also consider liver biopsy, which can be very helpful in people with concomitant diseases such as overlap of PBC and autoimmune hepatitis or overlap of PBC and non-alcoholic steatohepatitis as examples. So if we consider there could be overlap disease, that certainly brings liver biopsy into the equation. Non-invasive test-measure fibrosis, such as FibroScan or Fibroshore, can be helpful to determine the extent of disease, but do not actually make the diagnosis. Initial therapy for all patients is very straightforward. It's weight-based ursodeoxycholic acid or UDCA, typically using a dose of 13 to 15 milligrams per kilogram orally, twice daily. Non-responders, which are patients with a persistent elevation of the alkaline phosphatase above the limits of normal after one year on therapy, and those without cirrhosis can be treated with combined therapy using obeticholic acid or OBA and UDC. If you're going to use combined therapy, keep this in mind. OBA must be used with caution in patients with advanced fibrosis. Finally, pruritus. Pruritus is managed based on the severity of symptoms. Mild disease can be managed with skin creams, moisturizers, and more advanced disease, more advanced pruritus may need therapies such as colostyramine, sertraline, rifampin, and in rare cases naltrexone. And finally, long-term monitoring includes liver biochemical and function tests every three to six months, vitamin A and D levels, and PT or proforma time every two years. And in those patients with advanced disease, screening for esophageal viruses and hepatocellular carcinoma in patients with cirrhosis. Complications of PVC, and we'll finish on this note, include metabolic bone disease, hypercholesterolemia, fat malabsorption, and cirrhosis. Thank you for listening to our video tip of the week, and remember the annual GI advanced practice variety course is on March 31st and April 1st of this year. And once again, we are all in this together, GI physicians, GI APPs to build the optimal and successful GI APP teams with our simple goal of providing high quality care to our patients. We'll see you next week.

primary biliary cholangitis

autoimmune disease

environmental causes

symptoms

diagnosis