serum alkaline phosphatase is markedly raised in more than 95% of cases, and usually from an early stage
typically the serum ALP will be raised up to three or four times normal (1)
mild elevation of aminotransferases alanine aminotransferase [ALT] or aspartate aminotransferase [AST] - raised later rather than early
increased levels of immunoglobulins – mainly IgM
late features
serum bilirubin is raised in most cases
alterations in prothrombin and serum albumin
immunology:
anti-mitochondrial antibodies (specifically to the M2 antigen) occur in 95% of cases - these may rarely be seen in a small proportion of patients with chronic active hepatitis or cryptogenic cirrhosis
smooth muscle antibodies in 50%
antinuclear factor in 20%
IgM is raised in more than 80% of cases
negative ANCA
increased cholesterol levels
liver biopsy
confirms the diagnosis but not mandatory to make the diagnosis
useful for assessment of the activity and staging of the disease
stainable levels of copper may approach that seen in Wilson's disease as a result of the cholestasis.
imaging
MRI or endoscopic retrograde cholangiography – to exclude primary sclerosing cholangitis or other disorders that might lead to chronic cholestasis
transient elastography - to evaluate the degree of liver fibrosis,
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