Trivedi PJ, Bowlus CL, Yimam KK, et al. - Estimates of the incidence and prevalence of primary sclerosing cholangitis (PSC) vary, with most studies carried out in North America and Western Europe, with the latter showing a steady rise in disease occurrence over time.

• Of the 4,922 published studies, 17 met the inclusion criteria, with 16 documenting incidences and 14 documenting prevalence.

• The highest reported incidence of PSC was found in Northern Europe (Finland: 1.58 and Norway 1.3 per-100,000 population, respectively) and North America (Minnesota USA, 1.47), with the lowest found in the Mediterranean Basin (Italy, 0.1).

• The prevalence of the disease ranged from 31.7 in Finland and 23.99 in Minnesota, to 1.33 in Singapore and 0.0 in Alaska.

• A rise in disease incidence was detected across North America and Northern Europe (four studies), as well as an increase in prevalence over time (four studies), in studies that reported temporal occurrence.

• Nine of the included studies presented the incidence and hazards of clinical outcomes.

• In comparison to the control population, median transplant-free survival ranged from 9.7 years in the United States to 20.6 years in the Netherlands, with standardized mortality ratios of 2.5 and 4.2.

• The standardized cholangiocarcinoma incidence ranged from 235 in Finland to 398 in the Netherlands.