Primary biliary cirrhosis is a chronic progressive disease most often affecting women. It is characterized by the progressive destruction of the small intrahepatic bile ducts with portal inflammation leading to hepatic fibrosis and cirrhosis. Autoimmune diseases such as Sjogren's syndrome, rheumatoid arthritis, dermatomyositis, CREST syndrome, and autoimmune thyroiditis may be found in up to 80% of the patients. Primary biliary cirrhosis is also associated with renal diseases such as distal renal tubular acidosis, bacteriuria and glomerulopathy, but there is no case report of primary biliary cirhosis with renal manifestations in Korea. We are reporting a case of primary biliary cirrhosis with multiple renal abscess and IgA nephropathy with a review of the literature.(Korean J Hepatol 2000;6:223-228)
