Mortality in Autoimmune Liver Disease in Sweden: A Population-Based Cohort Study of 9,654 Patients

Epidemiological data on mortality in autoimmune liver diseases (AILDs) are scarce. We examined all-cause and cancer-related mortality in individuals with AILD from Sweden.

We identified 9654 individuals with AILD (3342 with autoimmune hepatitis (AIH), 3751 with primary biliary cholangitis (PBC), and 2561 with primary sclerosing cholangitis (PSC)) using national Swedish registries between 2001 and 2020. These were matched with 80 685 comparators from the general population at a ratio of 1:10 on age, sex, year of diagnosis and municipality. Rates of outcomes were estimated using Cox regression models, adjusted for matching factors and cardiovascular disease, diabetes, inflammatory bowel disease, chronic obstructive pulmonary disease, and education.

Individuals with AILD had higher mortality than comparators (adjusted hazard ratio (aHR) = 2.3, 95% CI = 2.2-2.4) and higher rates of cancer-related death (aHR = 2.1, 95% CI = 1.9-2.3). The presence of liver cirrhosis in AILD was related to even higher mortality, with aHR 5.8 (95% CI = 5.1-6.6). Both males and females with AILD had increased mortality (males aHR = 2.6, 95% CI = 2.4-3.0, and females aHR = 2.2, 95% CI = 2.1-2.3). The mortality was higher in individuals aged 18-50 years (aHR = 4.6, 95% CI = 3.6-5.8), than in individuals above 50 years (aHR = 2.2, 95% CI = 2.1-2.3). Overall mortality rates and cancer-related death were particularly high in individuals with PSC compared to their matched comparators, with aHR = 4.1 (95% CI = 3.2-5.2) and aHR = 6.4 (95% CI = 4.0-10.3), respectively.

Patients with AILDs have increased rates of overall and cancer-related mortality compared to matched comparators, and relative risks are highest in cirrhosis, younger age and PSC.