Update on primary biliary cirrhosis.
Primary biliary cirrhosis is an autoimmune chronic liver disease characterized by progressive bile duct destruction eventually leading to cirrhosis, liver failure, and death. The autoimmune pathogenesis is supported by a plethora of experimental and clinical data, such as the presence of autoreactive T cells and serum autoantibodies. The aetiology remains unknown, although evidence suggests a role for both genetic susceptibility and environmental factors that remain to be determined. In fact, a number of chemicals and infectious agents have been proposed to induce the disease in predisposed individuals. The recent availability of several murine models will significantly help in understanding pathophysiology mechanisms. In this review, we critically summarize the most recent data on the aetiopathogenesis of primary biliary cirrhosis, discuss the latest theories and developments, and suggest directions for future research.
Invernizzi
Pietro
P
Division of Internal Medicine and Hepatobiliary Immunopathology Unit, IRCCS Istituto Clinico Humanitas, Rozzano, Italy. pietro.invernizzi@humanitas.it
Selmi
Carlo
C
Gershwin
M Eric
ME
D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D015551:Autoimmunity; D018450:Disease Progression; D020022:Genetic Predisposition to Disease; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology*; D008105:Liver Cirrhosis, Biliary / Q000209:etiology*; D012307:Risk Factors; D013601:T-Lymphocytes / Q000276:immunology
D001323:Autoantibodies