Treatment of primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liv er, which predominantly affects middle age women. Most patients are diagnos ed when asymptomatic. The disease is characterised by chronic. granulomatou s inflammation of the small bile ducts, which leads to progressive ductopen ia, cholestasis, fibrosis, cirrhosis and eventual liver failure. All PBC pa tients with abnormal liver biochemistry should be considered for therapy. U rsodeoxycholic acid (URSO) treatment reduces intracellular hydrophobic bile acid levels and thereby may have a cytoprotective effect on cell membranes . URSO may also act as an immun-modulating agent. Multicenter randomised co ntrolled trials proved that the treatment is associated with a marked impro vement in serum biochemical markers of cholestasis, i.e. bilirubin, ALP, GG T, including fall in serum cholesterol levels. Treatment does not seem to b enefit the symptoms of fatigue, pruritus, and osteoporosis. UDCA has been s hown when given in a dose of 15mg/kg daily for up to 4 years to prolong the time to liver transplantation or death. Immunosuppressive therapy: based o n the immunological abnormalities, several immunosuppresive drugs have been tested. Neither azathioprine nor cyclosporine was found in large enough tr ials to show beneficial effect on survival. D-penicillamine, cholchicin, me thotrexat, prednisolone were round without significant long-term benefit. C ombination therapy with URSO and budenoside appears to add some benefit to URSO monotherapy, but further studies are needed. Liver transplantation. Th e most crucial question is the timing. Serum bilirubin, Mayo risk score and some other factors such as uncontrollable pruritus and severe osteoporosis influence the decision. Recurrence of PBC in allograft is rare, the progre ss is slow. and is no reason for not recommending transplantation. Symptoma tic treatment of pruritus, sicca syndrome and preventive treatment of osteo porosis. neuropathy and fat soluble vitamin deficiency is also important. ( C) 2001 Elsevier Science Ltd. All rights reserved.