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Autoimmune

Primary biliary cholangitis disease course, prognosis vary greatly

Posted on July 22, 2021

The primary biliary cholangitis (PBC) is an autoimmune liver disease that has varying courses with some patients rapidly reaching end-stage liver disease and some remaining asymptomatic for years. The life expectancy of patients with PBC is shorter than the general population.

According to RethinkPBC, disease progression typically starts with positive antimitochondrial antibodies before progressing to cholestasis or inflammation and fibrosis. A severe form of PBC found in up to 30% of patients can result in the early development of liver fibrosis and failure. The final stage of PBC is cirrhosis, often leading to advanced complications, such as end-stage liver disease and hepatic decompensation).

One study found that 31% of patients at Stage 1 at the time of PBC diagnosis progressed to cirrhosis over 4 years.

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