Primary biliary cholangitis

How might primary biliary cholangitis be treated?

Treatment for primary biliary cholangitis (PBC) is life-long and aims to slow the disease progression and improve the symptoms that reduce quality of life. The first-line treatment for PBC is long-term use of ursodiol, also called ursodeoxycholic acid (UDCA). This medication, which is approved by the Food and Drug Administration (FDA) for the treatment of PBC, has been shown to slow disease progression and reduce the need for a liver transplant. Liver function should be monitored periodically to help detect people who do not adequately respond to UDCA. Because UDCA therapy does not improve fatigue, itching (pruritus), bone problems, and many other signs and symptoms of PBC, these should be addressed separately.

Obeticholic acid (OCA), a second-line treatment for PBC, is also approved by the FDA. OCA may be used along with UDCA in people who have an inadequate response after at least a year of treatment. It may also be used as a single treatment in people who cannot tolerate UDCA. OCA is used to increase the flow of bile from the liver. Studies investigating the effect that OCA has on survival of people with PBC are ongoing, but data suggest that its use in combination with UDCA may lower the risk for liver complications and liver-related deaths. OCA is not recommended in people with PBC who have advanced liver disease (decompensated cirrhosis). Like UCDA, OCA typically does not improve symptoms of PBC.

Fibrates, medications currently approved by the FDA as lipid-lowering medications, are being studied as a promising new drug for PBC and may improve liver function as well as pruritus. Like OCA, the use of fibrates is also discouraged in people with decompensated liver disease.

Because the symptoms of PBC do not typically improve with UDCA or OCA treatment, they are treated separately. Management of pruritus may include lifestyle modifications (such as avoiding tight clothing and using moisturizers) and/or medications such as cholestyramine.

Liver transplantation may be successful in people who have the transplant before liver failure occurs.

Details regarding treatment guidelines for PBC are available from the American Association for the Study of Liver Diseases.

Last updated on 05-01-20

Name: The PBCers Organization 1430 Garden Road
Pearland, TX, 77581, United States
Phone: +1-346-302-1620 Email: pbcsite@pbcers.org Url: https://pbcers.org/
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: American Liver Foundation 39 Broadway, Suite 2700
New York, NY, 10006, United States
Phone: +1-212-668-1000 Toll Free: +1-800-465-4837 (Helpline) Email: https://liverfoundation.org/for-patients/contact-us/ Url: https://liverfoundation.org/
Name: The PBC Foundation (UK) Ltd 2nd Floor 6 Hill Street
Edinburgh, EH2 3JZ, United Kingdom
Phone: + 44 (0) 131 556 6811 Email: info@pbcfoundation.org.uk Url: https://www.pbcfoundation.org.uk/

Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.

Drug Name Generic Name
Ocaliva obeticholic acid

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