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Summary of stagesStage 1 - Portal Stage: Normal sized triads; portal inflammation, subtle bile duct damage. Granulomas are often detected in this stage. Stage 2 - Periportal Stage: Enlarged triads; periportal fibrosis and/or inflammation. Typically characterized by the finding of a proliferation of small bile ducts. Stage 3 - Septal Stage: Active and/or passive fibrous septae. Stage 4 - Biliary Cirrhosis: Nodules present; garland or jigsaw pattern. EtiologyThe cause of the disease is unknown at this time, but research indicates that there is an immunological basis for the disease, making it an autoimmune disorder. Most of the patients (>90%) seem to have auto-mitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC-E2), an enzyme complex that is found in the mitochondria. In addition, a more specific test to confirm this disease from a bone disorder such as Paget's (disease of bone) which also has increases in Alkaline phosphatase is the Gamma-glutamyl trans peptidase test (GGTP). An increase in GGTP could indicate presence of Primary Biliary Cirrhosis.TherapyThere is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. However, specific treatment for fatigue, which may be invalidating in some patients, is unavailable. Ursodeoxycholic acid (Ursodiol) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (liver function tests). It has a minimal efect of symptoms and whether it improves prognosis is controversial. To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, cholestyramine (a bile acid sequestrant) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. As in all liver diseases, alcoholic beverages are contraindicated. In advanced cases, a liver transplant, if successful, results in a favourable prognosis.from: http://en.wikipedia.org/wiki/Biliary_cirrhosis#Signs_and_symptoms