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

## TL;DR

This study shows that people with primary biliary cholangitis have higher mortality than the general population, whether or not they have cirrhosis.

## Contribution

The study demonstrates that PBC increases mortality risk regardless of cirrhosis presence, using nationwide data and matched population comparators.

## Key findings

- PBC patients with cirrhosis had a 2.41-fold higher 10-year mortality risk compared to matched comparators.
- Non-cirrhotic PBC patients had an 18.5% 10-year mortality risk versus 14.3% in the general population.
- The 10-year HCC risk for cirrhotic PBC patients was 2.6%.

## Abstract

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.

Primary biliary cholangitis (PBC) is a rare chronic liver disease which can cause development of liver cirrhosis, but its impact on survival is poorly understood, particularly for those without liver cirrhosis. We compared mortality risk between Danish patients with PBC and the general population, matching them on sex and age. We found that patients with PBC without cirrhosis had a slightly higher mortality risk than the general population (18.5% vs. 14.3% after 10 years), but the difference may not be attributable to PBC alone.