Managing Primary Biliary Cholangitis (PBC)

Consistently elevated alkaline phosphatase (ALP) may increase patients' risk of disease progression and poor outcomes1

Increased ALP has been associated with2,3:

  • Cholestasis

  • Bile duct destruction

  • Disease progression

ALP <1.67 x upper limit of normal (ULN) was associated with1*:

  • Improved outcomes

  • Longer transplant-free survival

Based on a meta-analysis of 4845 patients that showed a log-linear association between ALP levels and the risk of liver transplantation and death after 1 year and up to 5 years of follow-up.1

According to the AASLD guidelines, 2 prognostic models have been used to estimate the risk of death or liver transplant for patients with PBC4:

GLOBE score4*:

  • Developed from a retrospective cohort of 2488 ursodeoxycholic acid (UDCA)–treated patients and validated by a second cohort of 1631 European and North American patients

  • Included 5 variables: serum bilirubin, albumin, ALP, platelet count after 1 year of treatment, and age at start of therapy

  • Patients with a score >0.30 had a shorter transplantation-free survival than an age- and sex-matched healthy population

UK-PBC score4*:

  • Developed from a cohort of 3165 patients

  • Found that serum ALP, aminotransferases, and bilirubin after 12 months of therapy—as well as albumin and platelets at baseline—predicted the risk of a liver transplant or liver-related death occurring within 5, 10, or 15 years

AASLD=American Association for the Study of Liver Diseases.

The GLOBE and UK-PBC scores are superior to prior models, although validation in other ethnic groups and populations is needed.

PBC-related pruritus can be extremely debilitating and may impact patients' daily life5-7

  • Up to 70% of patients with PBC experience pruritus5

  • Pruritus may have a pronounced impact on daily life5,8

  • There is a need for new, approved treatment options to effectively manage PBC-related pruritus5,8

References: 1. Lammers WJ, van Buuren HR, Hirschfield GM, et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterology. 2014;147(6):1338-1349.e5. doi:10.1053/j.gastro.2014.08.029 2. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67(1):145-172. doi:10.1016/j.jhep.2017.03.022 3. Levy C, Manns M, Hirschfield G. New treatment paradigms in primary biliary cholangitis. Clin Gastroenterol Hepatol. 2023;21(8):2076-2087. doi:10.1016/j.cgh.2023.02.005 4. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69(1):394-419. doi:10.1002/hep.30145 5. Hegade VS, Mells GF, Fisher H, et al. Pruritus is common and undertreated in patients with primary biliary cholangitis in the United Kingdom. Clin Gastroenterol Hepatol. 2019;17(7):1379-1387.e3. doi:10.1016/j.cgh.2018.12.007 6. Mells GF, Pells G, Newton JL, et al. Impact of primary biliary cirrhosis on perceived quality of life: the UK-PBC national study. Hepatology. 2013;58(1):273-283. doi:10.1002/hep.26365 7. Hegade VS, Kendrick SFW, Rehman J, Jones DEJ. Itch and liver: management in primary care. Br J Gen Pract. 2015;65(635):e418-e420. doi:10.3399/bjgp15X685477 8. Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018;67(9):1568-1594. doi:10.1136.gutjnl-2017-315259