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PBC Pruritus FAQs
FAQs Regarding the Management of Pruritus in PBC

Released: December 23, 2024

Expiration: December 22, 2025

Stuart C. Gordon
Stuart C. Gordon, MD
Marlyn J. Mayo
Marlyn J. Mayo, MD
Pam Rivard
Pam Rivard, RN
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Key Takeaways
  • In PBC, pruritus may appear or worsen even before diagnosis and even if liver enzyme levels are normal.

At a recent symposium, 2 experts and a person living with primary biliary cholangitis (PBC) addressed frequently asked questions about managing pruritus in PBC. Read on to learn what they had to say to improve the care of people living with this debilitating condition.

Can patients with PBC experience pruritus even when liver enzyme levels are normal? If so, should a biopsy be performed to confirm the diagnosis of PBC and to confirm that the pruritus is PBC related?

Stuart C. Gordon, MD: Yes, people with PBC can experience pruritus with normal liver enzymes. In my experience, I have not observed a strong correlation between itch and biochemical abnormalities. However, other tests would be needed to corroborate cholestatic itch.

Marlyn J. Mayo, MD: Independent of itch, if you follow the American Association for the Study of Liver Diseases guidelines for diagnosing PBC, a diagnosis can be made if 2 of these 3 criteria are met: elevated alkaline phosphatase levels, presence of PBC-related antibodies, or histologic evidence. So, if a person is experiencing itch, but their liver enzyme levels are normal, a liver biopsy should be recommended to rule out other liver diseases.

For the faculty member who is living with PBC, at what point in your diagnosis did your itching start, and did it correlate with bilirubin or liver enzyme levels? 

Pam Rivard, RN: My itching started years before my diagnosis. I would have ongoing discussions about possible explanations for the persistent itching with my primary care provider (PCP): “Is it my gallbladder?” “Is it my liver?” And the tests would come back normal.

One day, my PCP called me in a panic because my routine liver lab test results were very abnormal. I had suspected for decades that something was wrong with my liver. However, by the time I was diagnosed with PBC (after exhibiting high liver labs), I was no longer experiencing itch.

More recently, during the past 2 years, my liver enzymes remained high, but the severity of my itch did not increase until recently. I’ve found that my pruritus symptoms have been inconsistent with my liver lab tests over the past 2 decades. Historically, in my case, the 2 variables have been unrelated.

How should people with cirrhosis and PBC-associated pruritus be treated? 

Marlyn J. Mayo, MD: Agents such as rifampin and bile acid–binding resins, although given off-label, are not contraindicated in people with cirrhosis and may be helpful. As long as the patient understands the risks and can be monitored closely, I feel comfortable prescribing these medications.

Seladelpar and elafibranor are not approved for use in people with decompensated cirrhosis, but they can be prescribed for those with mild hepatic impairment. PPAR agonists can be used to treat pruritus in PBC patients with Child-Pugh class A cirrhosis who also have an indication for second line therapy, such as intolerance or inadequate biochemical response to ursodiol. Ileal bile acid inhibitors may be an option in the future, but we will need to see more evidence of their efficacy in patients with cirrhosis, specifically.

Stuart C. Gordon, MD: Oral cholestyramine remains safe for these patients, and the newer PPAR agonists represent promising options in this situation. Fibrates can raise concerns in this population.

How can healthcare professionals treat itch in people with non-PBC liver diseases?

Stuart C. Gordon, MD: I prefer trying bile acid–binding resins at the outset. Notably, people with other liver diseases complain of itch, including those with metabolic and alcohol related liver disease. Therefore, itch is not unique to those with cholestatic liver disease. I would hope that there may be a future role for PPAR agonists in these cases. Though they would be prescribed off-label, itch is a very debilitating symptom that is not limited to cholestatic liver disease.

Your Thoughts?
How do you approach treatment for pruritus in patients with decompensated cirrhosis who itch? Get involved in the discussion by posting a comment below.