Epidemiology and liver transplantation burden of primary biliary cholangitis: a retrospective cohort study (original) (raw)

rimary biliary cholangitis (PBC) is a rare chronic autoimmune cholestatic liver disease characterized by destruction of the small intrahepatic bile ducts. It predominantly affects middle-aged and elderly women. 1,2 Despite its rarity, PBC is an important cause of liver-related morbidity. 2-5 In the United States, the annual economic burden of PBC has been estimated to be 69−69-69−115 million. 3 There is concern that the economic impact of PBC could increase significantly in the future because of the increasing incidence and prevalence of this disease; 6-12 however, with improved treatment options, the costs may be attenuated. 13-15 The epidemiology of PBC was first described as early as 40 years ago, 16 although decades later no study has yet investigated the national prevalence of PBC in Canada. A number of studies have examined the epidemiology of PBC globally, illustrating considerable variability in the prevalence of the disease; prevalence rates range from 6.7 to 402 cases per million, while incidence rates range from 0.7 to 58 cases per million. 1 A growing number of studies suggest that the incidence and prevalence of PBC are increasing. 6-12 The driver of this growth remains unclear, but it may be the result of an increase in incidence, longer survival and