Background: 3-hydroxy-3-methylglutaryl coenzyme A-reductase inhibitor as well as fibrate, lipid-lowering drugs, recently has been reported to reduce disease markers of primary biliary cirrhosis (PBC). Case Report: We report the long-term observation of a case of PBC treated sequentially with several lipidlowering drugs and the effects of these drugs on the liver function tests. A 39-year-old female was diagnosed as PBC because of liver dysfunction accompanying high levels of immunoglobulin M (IgM), positive antimitochondrial antibody (AMA), and histological findings as stage II of PBC. She was also diagnosed as familial hypercholesteremia (IIa) because of her family history. She was treated with ursodeoxycholic acid (UDCA), which led to a transient improvement in liver function tests. Consequently, she was sequentially given several lipid-lowering drugs, probucol, bezafibrate, pravastatin and atorvastatin in addition to UDCA because of high levels of cholesterol. Bezafibrate, pravastatin, and atrovastatin, but not probucol, reduce disease markers of PBC. Particularly, long-term treatment with atorvastatin for more than 2.5 years led to continuously improving most significantly, not only the colesterol levels, but also liver function tests including IgM and AMA, compared to the other lipid-lowering drugs used throughout the clinical course. There were no side effects throughout the treatment. Conclusions: Our case indicates that statins, as well as fibrates, could ameliorate PBC in the long-term without side effects, suggesting that statins may be a first-line treatment for PBC particularly in patients with hypercholesteremia.

Keywords: PBC, HMG-CoA reductase inhibitors, Statin, fibrates, Probucol, Multiple drug-resistant gene (MDR), Phospholipid, Ursodeoxycholic acid (UDCA)