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The diagnosis is predominantly based on chronic elevation of alkaline phosphatase and the presence of anti\u2010mitochondrial antibodies or other specific antinuclear antibodies (i.e. anti\u2010gp210 and anti\u2010sp100). Even in early\u2010stage disease, health\u2010related quality of life can be severely impaired by symptoms such as pruritus, fatigue, and sicca syndrome and metabolic bone disease should be assessed and treated. The prognosis of the disease is, however, largely determined by the development of cirrhosis and its complications. Ursodeoxycholic acid is associated with an improved prognosis and should be initiated and continued in all patients. Clinical outcome is related to the biochemical response to ursodeoxycholic acid, but the prognosis of those with an incomplete response is still better than those who remain untreated. 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