A clinical history of primary biliary cholangitis (PBC)

Explore some key dates in the clinical history of PBC, from its beginnings to today’s changing landscape.


Addison and Gull are first to describe prolonged obstructive jaundice with patent bile ducts1


Dauphin and Sinclair coin the term “primary biliary cirrhosis” (PBC)2


Ahrens and colleagues expand the definition of PBC to be a disease that affects middle-aged to older women with progressive jaundice, pruritus, and hepatosplenomegaly1


Sheila Sherlock portrays 42 cases of PBC that she personally followed, which are instrumental in establishing PBC as an autoimmune disorder3


Rubin and colleagues describe the fundamental histologic lesion of PBC4


cDNA for major mitochondrial antigen of PBC is identified as pyruvate dehydrogenase complex5


Actigall (ursodeoxycholic acid) is launched with an initial indication of nonsurgical treatment of gallstones6


PBC becomes one of the leading indications for liver transplant7


Urso 250 and Urso Forte (ursodeoxycholic acid) tablets, indicated for the treatment of PBC, launch in the US8


Today—research is ongoing in the field of PBC

While PBC is rare, it is the most common chronic cholestatic liver disease in adult women.9

PBC—increasing recognition may lead to earlier diagnosis and treatment worldwide.

  • Incidence: 0.7 to 49 per million10,a
  • Prevalence: 6.7 to 402 per million10,a
  • Since 1988 PBC has been the second leading cause of liver transplant in women in the United States, behind hepatitis C11
  • In Denmark, 1.3% of deaths from cirrhosis are attributed to PBC12
  • In Europe, some researchers believe the incidence of PBC is rising as evidenced by
    • A change from 5.8 to 20.5 cases per million people per year in Sheffield, UK, between 1980 and 199913
    • A change from 12 to 17 cases per million people per year in Finland between 1988 and 199913,14

aVaries geographically.

References:  1. Poupon R. Primary biliary cirrhosis: a 2010 update. J Hepatol. 2010;52(5):745-758. doi:10.1016/j.jhep.2009.11.027.   2. Dauphinee JA, Sinclair JC. Primary biliary cirrhosis. Can Med Assoc J. 1949;61(1):1-6.  3. Heathcote EJ. Primary biliary cirrhosis: historical perspective. Clin Liver Dis. 2003;7(4):735-740. doi:10.1016/S1089-3261(03)00106-5.  4. Rubin E, Schaffner F, Popper H. Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Am J Pathol. 1965;46(3):387-407.  5. Yeaman SJ, Fussey SPM, Danner DJ, James OFW, Mutimer DJ, Bassendine MF. Primary biliary cirrhosis: identification of two major M2 mitochondrial autoantigens. Lancet. 1988;1(8594):1067-1070. doi:10.1016/S0140-6736(88)91894-6.  6. Ciba-Geigy Actigall detailing features laptop computers. The Pink Sheet. https://www.pharmamedtechbi.com/publications/the-pink-sheet/50/041/cibageigy-actigall-detailing-features-laptop-computers. Published October 10, 1988. Accessed August 20, 2015.  7. Tzakis AG, Carcassonne C, Todo S, Makowka L, Starzl TE. Liver transplantation for primary biliary cirrhosis. Semin Liver Dis. 1989;9(2):144-148. doi:10.1055/s-2008-1040506.  8. Urso 250/Urso Forte [package insert]. Birmingham, AL: Axcan Pharma US; 2004.  9. Primary biliary cirrhosis (PBC). Merck Manual website. http://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/fibrosis-and-cirrhosis/primary-biliary-cirrhosis-pbc#Etiology. Updated July 2013. Accessed August 20, 2015.  10. Hohenester S, Oude-Elferink RPJ, Beuers U. Primary biliary cirrhosis. Semin Immunopathol. 2009;31(3):283-307. doi:10.1007/s00281-009-0164-5.  11. Organ Procurement and Transplantation Network. US Health Resources and Services Administration website. http://optn.transplant.hrsa.gov/converge/latestData/rptData.asp. Updated May 22, 2015. Accessed August 20, 2015.  12. Sørensen HT, Thulstrup AM, Mellemkjar L, et al. Long-term survival and cause-specific mortality in patients with cirrhosis of the liver: a nationwide cohort study in Denmark. J Clin Epidemiol. 2003;56(1):88-93. doi:10.1016/S0895-4356(02)00531-0.  13. Blachier M, Leleu H, Peck-Radosavljevic M, Valla D-C, Roudot-Thoraval F. The burden of liver disease in Europe: a review of available epidemiological data. J Hepatol. 2013;58(3):593-608. doi:10.1016/j.jhep.2012.12.005.  14. Rautiainen H, Salomaa V, Niemelä S, et al. Prevalence and incidence of primary biliary cirrhosis are increasing in Finland. Scand J Gastroenterol. 2007;42(11):1347-1353. doi:10.1080/00365520701396034.