Natural history and management of primary biliary cirrhosis

tural history and management of primary biliary cirrhosis Review (964) Total Article Views Authors: Al-Harthy N, Kumagi T Published Date December 2012 Volume 2012:4 Pages 61 - 71 DOI: http://dx.doi.org/10.2147/HMER.S25998 Received: 07 May 2012 Accepted: 11 September 2012 Published: 04 December 2012 Nadya Al-Harthy,1 Teru Kumagi2 1Gastroenterology and Hepatology, Royal Hospital, Muscat, Oman; 2Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, Japan Abstract: Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. It is a rare disease with prevalence of less than one in 2000. Its prevalence in developing countries is increasing presumably because of growth in recognition and knowledge of the disease. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The prognosis of PBC has improved because of early diagnosis and use of ursodeoxycholic acid, the only established medical treatment for this disorder. When administered at adequate doses of 13–15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. However, some patients do not respond adequately to ursodeoxycholic acid and might need alternative therapeutic approaches.