PBC is a chronic cholestatic liver disease characterized by high-titer serum antimitochondrial autoantibodies (AMAs) and autoimmune-mediated destruction of small- and medium-sized intrahepatic bile ducts [1–3]. The first description of biliary cirrhosis, albeit possibly secondary, can be traced back to the work of the Italian pathologist Giovanni Battista Morgagni from Padua in 1761; the first report of nonobstructive biliary cirrhosis was by Addison and Gull in 1851. Subsequently, the term PBC was accepted in the medical literature [4], and in 1959 Dame Sheila Sherlock described the first series of patients affected by PBC who had been followed over the previous decade and noted that patients presented with pruritus as well as the signs and symptoms of end-stage liver disease including jaundice [5].