Abstract
PBC remains a female predominant disease, but the disease is more prevalent in males than previously believed, with contemporary studies showing a 4-6:1 female-to-male ratio. Men present later in disease process, as evidenced by older age, higher bilirubin, lower platelet count, higher model for end-stage liver disease -Na, and lower UDCA exposure-time in several studies. Although males present at a more advanced stage of disease at diagnosis than females, male sex has been shown to be associated with a higher death or transplantation, or liver-related death or transplantation in PBC cirrhosis, even after adjusting for age, comorbidities, UDCA response, and the stage of disease.
A diagnosis of PBC is often delayed among males because of the myth that PBC is extremely rare in males. Greater research and education are needed to raise awareness, so that PBC is suspected and diagnosed at an earlier stage among males.
