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PRIMARY BILIARY CHOLANGITIS (PBC) Disease Progression

PBC is a disease with high patient burden1,2

PBC is an autoimmune liver disease commonly characterized by progression of cholestasis that can lead to hepatic complications and decompensation1,2

  • Life expectancy is worse than general population, but the course of disease and prognosis vary greatly2
  • Fatigue and pruritus are prevalent symptoms which have a major impact on quality of life2
  • Some patients rapidly advance to end-stage disease and liver transplantation at a young age, while others remain asymptomatic for decades3

PBC disease progression3-7

Sequence of PBC disease progression Sequence of PBC disease progression

Some patients can progress to end-stage disease rapidly8

As the presentation of PBC varies amongst patients, so does progression3

Image showing progression from PBC diagnosis to cirrhosis Image showing progression from PBC diagnosis to cirrhosis
  • a Data from an analysis of recorded histological stages from liver biopsies performed annually during a double-blind, placebo-controlled, randomized study in 222 PBC patients were analyzed using a Markov model to describe the time course of histological progression in PBC; of the patients at stage 1 at entry (n=15), 31% showed cirrhosis after 4 years.8
  • ALP, alkaline phosphatase; AMA, antimitochondrial antibody.

References:
1. Angulo P, Batts KP, Therneau TM, Jorgensen RA, Dickson ER, Lindor KD. Long-term ursodeoxycholic acid delays histological progression in primary biliary cirrhosis. Hepatology. 1999;29(3):644-647. 2. Lammers WJ, Kowdley KV, van Buuren HR. Predicting outcome in primary biliary cirrhosis. Ann Hepatol. 2014;13(4):316-326. 3. Younossi ZM, Bernstein D, Shiffman ML, et al. Diagnosis and management of primary biliary cholangitis. Am J Gastroenterol. 2019;114(1):48-63. 4. Selmi C, Bowlus CL, Gershwin ME, Coppel RL. Primary biliary cirrhosis. Lancet. 2011;377(9777):1600-1609. 5. Poupon R. Primary biliary cirrhosis: a 2010 update. J Hepatol. 2010;52(5):745-758. 6. 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. 7. Dyson JK, Hirschfield GM, Adams DH, et al. Novel therapeutic targets in primary biliary cirrhosis. Nat Rev Gastroenterol Hepatol. 2015;12(3):147-158. 8. Locke GR III, Therneau TM, Ludwig J, Dickson ER, Lindor KD. Time course of histological progression in primary biliary cholangitis. Hepatology. 1996;23(1):52-56.