title: Diagnosis of primary biliary cirrhosis.
abstract: Primary biliary cirrhosis is the archetypal autoimmune liver disease, with the disease label describing a chronic granulomatous lymphocytic small bile duct cholangitis, which now most commonly presents asymptomatically and at an early pre-cirrhotic stage. Disease is more common than thought, with 1 in 1000 women over the age of 40 affected. Characteristic immunologic features of the disease assist clinicians in ready non-invasive diagnosis of patients, even if asymptomatic with only anicteric/cholestatic liver biochemical profiles. Over 90% of patients are anti-mitochondrial antibody positive, and for those negative, a significant proportion have highly specific anti-nuclear antibody profiles. Liver biopsy remains useful in certain settings where clarity is needed to confirm diagnosis, exclude alternative disease, and assess the relative contribution of PBC to other co-existent liver injury, and seeks to demonstrate in particular the classic bile duct lesions, as well as the degree of interface activity.
authors: Department of Medicine, University of Toronto, Canada. gideon.hirschfield@uhn.on.ca
authors: Gideon M
authors: GM
authors: Hirschfield
mesh_terms: D000469:Alkaline Phosphatase / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D001706:Biopsy; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D008928:Mitochondria / Q000276:immunology; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D000974:Antibodies, Antinuclear; D005723:gamma-Glutamyltransferase; D000469:Alkaline Phosphatase