Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease that is the cause of 1-2% of deaths from cirrhosis and constitutes the third most common indication for liver transplantation in adults.
The name of this disease was changed from primary biliary cirrhosis to primary biliary cholangitis in 2014 11.
The typical patient is a middle-aged woman presenting with symptoms of fatigue and pruritus and laboratory test evidence of cholestasis.
Clinical features include 12:
- pruritus (often the first symptom)
- skin hyperpigmentation, especially of arms and trunk
- xanthomata and xanthelasma due to hypercholesterolemia
- interestingly hypercholesterolemia does not confer an increased risk of death from atherosclerosis
- right upper quadrant abdominal discomfort
PBC is characterized by the destruction of small intrahepatic bile ducts, portal inflammation, and progressive scarring. The cause of PBC is unknown, but it is probably due to an inherited abnormality of immunoregulation.
Serum anti-mitochondrial antibody (AMA) tests are highly sensitive and specific for PBC (85-100%10).
- cholelithiasis (~40%) 8
- other autoimmune diseases, e.g. Sjögren syndrome (~75%), autoimmune thyroiditis (~25%), systemic sclerosis (~10%) 5,12
- interstitial lung disease: ~15% 6
hepatocellular carcinoma develops in ~5% 7
- additional risk in older patients, males, history of diabetes mellitus or past hepatitis B virus infection 7
- hepatic osteodystrophy 9
- T2: if parenchymal lace-like fibrosis and periportal halo sign are seen together the sensitivity for PBC can approach 70% 2
Other features detectable on MRI include:
- periportal hyperintensity (cuffing)
- segmental hypertrophy, notably of the caudate lobe
- hepatic surface irregularity due to regenerative nodules
- regional lymphadenopathy: tends to dominate in the gastrohepatic ligament and porta hepatis 4
Treatment and prognosis
Ursodeoxycholic acid is the mainstay for medical management 12. In cases of PBC that advance despite pharmacotherapy, liver transplantation clearly improves the survival and quality of life.
PBC can recur after transplantation but with a frequency much lower than that for hepatitis and other diseases.
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- 6. Shen M, Zhang F, Zhang X. Primary biliary cirrhosis complicated with interstitial lung disease: a prospective study in 178 patients. J. Clin. Gastroenterol. 2009;43 (7): 676-9. doi:10.1097/MCG.0b013e31818aa11e - Pubmed citation
- 7. Rong G, Wang H, Bowlus CL et-al. Incidence and risk factors for hepatocellular carcinoma in primary biliary cirrhosis. Clin Rev Allergy Immunol. 2015;48 (2-3): 132-41. doi:10.1007/s12016-015-8483-x - Pubmed citation
- 8. Bayless TM, M.D. AMD. Advanced Therapy in Gastroenterology and Liver Disease. pmph inc. ISBN:1550092480. Read it at Google Books - Find it at Amazon
- 9. López-Larramona G, Lucendo AJ, González-Castillo S et-al. Hepatic osteodystrophy: An important matter for consideration in chronic liver disease. World J Hepatol. 2011;3 (12): 300-7. doi:10.4254/wjh.v3.i12.300 - Free text at pubmed - Pubmed citation
- 10. Dähnert W. Radiology Review Manual. LWW. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 11. Beuers, U., Gershwin, M. E., Gish, R. G., Invernizzi, P., Jones, D. E., Lindor, K., ... & Vierling, J. M. (2015). Changing nomenclature for PBC: from ‘cirrhosis’ to ‘cholangitis’. Hepatology, 62(5), 1620-1622. doi:10.1038%2Fajg.2015.312
- 12. Fauci AS. Harrison's principles of internal medicine. New York: McGraw-Hill, Medical Publishing Division; 2008.