What is new in primary biliary cirrhosis?

Abstract

Primary biliary cirrhosis is a chronic cholestatic liver disease characterized by immune-mediated destruction of the small- and medium-sized intrahepatic bile ducts. The disease mainly affects middle-aged women who frequently suffer from concomitant autoimmune diseases. This review discusses the most recent findings in the epidemiology, natural history and approaches used for the accurate diagnosis of the disease. We critically review emerging data reporting on the role of genetics such as those of genome-wide association studies. Special attention is given to recent studies reporting on the immunopathogenesis of the disease and the role of plasma cells, apotopes, immunosenescence, autophagy and the involvement of components of the innate immunity in the induction of this enigmatic disease.