Advances in Treatment of Primary Biliary Cholangitis (PBC) - Episode 7

Care Management in PBC

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Kris Kowdley, MD, FACP, FACG, AGAF, FAASLD, reviews the changes in the screening and diagnosis of primary biliary cholangitis in the recently published CDLF guidelines, as well as when a primary care physician should refer a patient to a specialist to assess disease presence.

Kris Kowdley, MD, FACP, FACG, AGAF, FAASLD: There are some important differences on the recent update that we provided for management and diagnosis of PBC [primary biliary cholangitis]. We talk about early recognition for patients who may benefit from second-line treatment. Don’t wait for the alkaline phosphatase to be over 1.67, or over 2 times the upper limit of normal, but consider that for patients who have an alkaline phosphatase greater than 1.5 times the upper limit of normal. We also talk about the fact that bilirubin levels should be monitored. Maybe there’s a lower threshold for bilirubin—0.6 mg/dL might be a criterion at which patients should be considered for second-line treatment.

We’ve also emphasized the use of noninvasive testing, whether it’s using transient elastography, MR [magnetic resonance] elastography, or other noninvasive tools to identify which patients may be at risk for progression. Additionally, those may be patients who would be candidates for earlier intervention with second-line treatment. Finally, a third-line treatment or combinations of different medications as they become available—to aim at true normalization or achieving a biochemical remission—is also important.

About referrals, any patient with a confirmed positive antimitochondrial antibody [AMA] should be referred for further consideration. Any patient with elevated alkaline phosphatase—that isn’t obviously related to bone or another cause—should be referred. One thing that happens is alkaline phosphatase isn’t routinely measured and tested in patients who may not have symptoms. But if you identify a patient who is a woman who has hypothyroidism and a family history of autoimmune diseases, any elevation of alkaline phosphatase could represent PBC. If we don’t screen for the disease, we’re not going to find it because the majority of patients are asymptomatic but still may be at risk for progression.

Steven Flamm, MD, FAASLD, FACG: In general, patients should be referred from primary care to a gastroenterologist when they have an abnormal liver panel, because there are a lot of nuances that a specialist can ferret out to make an appropriate diagnosis. They don’t always have to be seen on an ongoing basis by a specialist, but if patients have an abnormal liver panel, it’s a good idea to have a specialist look at the patient.

Kris Kowdley, MD, FACP, FACG, AGAF, FAASLD: Do any of you want to add to that?

Edward Mena, MD: No, I agree with what Steve said. Abnormal liver enzymes and then refer.

David Victor III, MD: I don’t think you need to wait for symptomatology for a referral, even if it feels like just an abnormal lab. Oftentimes, it’s the subtlety of reading the tea leaves for these patients that leads to positive outcomes.

Kris Kowdley, MD, FACP, FACG, AGAF, FAASLD: Sonal, any comments about how to educate primary care physicians or internal medicine doctors to refer patients to us?

Sonal Kumar, MD, MPH: I agree. One of the big things we offer these patients is staging of their disease, which isn’t easy to do in primary care offices. So, it’s important that they see us.

Steven Flamm, MD, FAASLD, FACG: One other pearl for primary care offices and gastroenterologists: if a patient has chronic liver disease of any type and thrombocytopenia, the provider should be very worried that the patient has cirrhosis—whatever the liver disease, including primary biliary cholangitis. Keep in mind, the liver panel doesn’t have to have significant abnormalities. It can be a well-preserved liver panel. But if you see thrombocytopenia in a patient with chronic liver disease, you should be very concerned about cirrhosis.

Kris Kowdley, MD, FACP, FACG, AGAF, FAASLD: The most important take-home message is not to wait for symptoms or for patients to present with jaundice and ascites. Screen patients. Obtain appropriate serologic testing with ANA [antinuclear antibody] and AMA. If the patient is positive, refer the patient so we can both assess them—noninvasively, at the point of care—and stage their disease.

Transcript edited for clarity