This chronic inflammatory disease of the liver is characteristically associated with circulating autoantibodies and high serum immunoglobulin concentrations. Untreated, it progresses to cirrhosis, but the condition responds well to immunosup pressives. Some 80% will benefit from prednisolone which should be continued in the long term, as most patients relapse if the drug is withdrawn. Azathioprine (1 mg/kg daily) is effective as a steroid sparing agent, and usually permits reducing of prednisolone to 5-10 mg/d. Increasing azathioprine to 2 mg/kg allows further reduction in prednisolone dose but haematological toxicity may result and the blood count must be monitored every 2 months.
PRIMARY BILIARY CIRRHOSIS (PBC)
This chronic cholestatic liver disease affects 1 in 4000 people in the United Kingdom. Pruritus is a common early symptom, and can be helped by colestyramine. Chronic cholestasis leads to malabsorption of fat-soluble vitamins, particularly vitamin D, and deficiency of which must be corrected to avoid osteomalacea.
The aetiology of PBC is unknown but high titres of antimitochondrial antibody in the majority suggest involvement of immune mechanisms. There is no effective treatment. Adverse effects outweigh benefits from prednisolone, but budesonide is currently under assessment as it is highly extracted by the liver and thus poorly available to the systemic circulation. Ursodeoxycholic acid 10-15 mg/kg/d improves biochemical liver function tests, but appears not to lengthen survival or prevent complications.
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