Primary Biliary Cholangitis Increases Mortality Irrespective of Presence or Absence of Cirrhosis

Primary biliary cholangitis (PBC) is an autoimmune liver disease whose effect on long-term survival remains unclear. We aimed to compare the prognosis of patients with biopsy-confirmed PBC with population comparators.

We used nationwide healthcare registries to include all Danish patients diagnosed with histologically confirmed PBC in 1998-2020. We estimated the prevalence of PBC and the incidence in 2016-2019. Dividing PBC by presence or absence of cirrhosis, we used the cumulative incidence function to estimate the risk of death and HCC. We included 5:1 age- and sex-matched population comparators.

We included 1163 PBC patients (88.1% women, median age at diagnosis = 59.7 years); those with cirrhosis were older (median 63.9 vs. 59.0). Patients had more comorbidity at diagnosis than comparators, especially connective tissue disease. On January 1st 2021, the prevalence of PBC was 22.3 per 100,000 population. The incidence rate in 2016-2019 was 2.80 (95% CI: 2.58-3.02) per 100,000 population per year. PBC patients with cirrhosis had a higher 10-year risk of death than their matched comparators, adjusted relative risk = 2.41 (95% CI: 1.89-3.09). For patients with non-cirrhotic PBC, the 10-year risk of death was 18.5% (95% CI: 15.5-21.7) versus 14.3% (95% CI: 13.0-15.6) for their comparators, adjusted relative risk = 1.22 (95% CI: 1.03-1.47). Patients with cirrhotic PBC had a 10-year risk of HCC at 2.6% (95% CI: 0.8-6.0).

Patients with PBC have a worse prognosis than the general population irrespective of the presence or absence of cirrhosis at the time of PBC diagnosis.