Last edited 07/2018 and last reviewed 08/2021

In the absence of treatment, PBC is a slowly progressive disease, with the majority of patients advancing 1 histologic stage every 2 years and the median survival of symptomatic and asymptomatic patients being 7.5 and 16 years, respectively

  • most asymptomatic patients will develop symptoms over the course of 4.5 to 17.8 years, and survival among PBC patients without symptoms is still worse than that of a similar control population

  • in addition to the risk of cirrhosis and its inherent complications, approximately 50% of PBC patients experience the symptoms of fatigue and pruritus

  • patients with cirrhosis from advanced PBC are subject to all the usual complications of cirrhosis, including hepatoma (hepatocellular carcinoma) development, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, and oesophageal variceal bleeding