Burden of primary sclerosing cholangitis in Sweden (2002–2020): Incidence, outcomes, healthcare utilization, and costs

BACKGROUND: There is limited real-world evidence on the economic burden associated with primary sclerosing cholangitis (PSC). This study evaluated the prevalence, incidence, baseline characteristics, long-term outcomes, healthcare resource use (HRU), and healthcare costs associated with PSC in Sweden. METHODS: Adults with PSC were identified in the Swedish National Patient Register from 2002 to 2020 using International Classification of Diseases, 10th revision codes for PSC (K83.0A) and/or cholangitis (K83.0) + inflammatory bowel disease (IBD) (K50/K51). The index date was defined as the date of the first (incident) PSC diagnosis. Patients were required to have a look-back period of ≥360 days (baseline) and a follow-up period of ≥30 days. Annualized mean HRU and healthcare costs (in 2021 euros) were calculated at baseline and during follow-up. RESULTS: Overall, 4213 incident patients with PSC were included (mean age 48.4 y; 56.8% male; 73.0% with IBD). At baseline, few patients had cirrhosis (4.2%), hepatobiliary or pancreatic cancers (4.6%), or had undergone liver transplantation (1.0%). Median duration of follow-up was 5.7 years. Outpatient visits, number of hospitalizations and filled prescriptions, and length of inpatient stay significantly increased from baseline to end of follow-up, with a 117% increase in annualized mean total healthcare costs from €9442 to €20,487 (p<0.0001), with hospitalization being a primary driver. In total, 935 patients (22.2%) died. The 10-year risk of any complication (any malignancy, cirrhosis, or liver transplantation) was 25.9% (95% CI 24.0-27.9). CONCLUSIONS: HRU and healthcare costs for patients with PSC in Sweden were substantial and significantly increased after diagnosis. Effective therapies are needed to reduce disease progression and economic burden.