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.
PBC is characterised 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. thyroiditis, systemic sclerosis 5
- 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
Liver transplantation clearly improves the survival and quality of life for patients with advanced PBC.
PBC can recur after transplantation but with a frequency much lower than that for hepatitis and other diseases.
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- 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