ANSWER 88
The liver function tests show a predominantly obstructive picture with raised alkaline
phosphatase and gamma-glutamyl transpeptidase, while cellular enzymes are only slightly
raised. The symptoms and investigations are characteristic of primary biliary cirrhosis, an
uncommon condition found mainly in middle-aged women. In the liver there is chronic
inflammation around the small bile ducts in the portal tracts. Hypercholesterolaemia, xan-
thelasmata and xanthomata are common. The dry eyes and dry mouth may occur as part
of an associated sicca syndrome. Itching occurs because of raised levels of bile salts, and
can be helped by the use of a binding agent such as cholestyramine which interferes with
their reabsorption. The presence of antimitochondrial antibodies in the blood is typical of
primary biliary cirrhosis. These antibodies are found in 95 per cent of cases.
Hypothyroidism might explain some of her symptoms but the normal thyroid-stimulating
hormone (TSH) level shows that her current dose of 150&g thyroxine is providing adequate
replacement. The thyroid antibodies reflect the autoimmune thyroid disease which is asso-
ciated with other autoantibody-linked conditions such as primary biliary cirrhosis.
The diagnosis is confirmed by a liver biopsy. This should only be carried out after an ultra-
sound confirms that there is no obstruction of larger bile ducts. Ultrasound will help to
rule out other causes of obstructive jaundice although the clinical picture described here
is typical of primary biliary cirrhosis. No treatment is known to affect the clinical course
of this condition.
- The pattern of liver enzyme abnormalities usually reflects either an obstructive or
hepatocellular pattern. - Symptoms such as itching have a wide differential diagnosis. Dealing with the under-
lying cause, wherever possible, is preferable to symptomatic treatment.