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.
Original language | English |
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Pages (from-to) | 701-12 |
Number of pages | 12 |
Journal | Best Practice & Research: Clinical Gastroenterology |
Volume | 25 |
Issue number | 6 |
DOIs | |
Publication status | Published - Dec 2011 |
Keywords
- Alkaline Phosphatase
- Antibodies, Antinuclear
- Autoimmune Diseases
- Biopsy
- Humans
- Liver Cirrhosis, Biliary
- Mitochondria
- gamma-Glutamyltransferase