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Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Jun 7, 2008; 14(21): 3313-3327
Published online Jun 7, 2008. doi: 10.3748/wjg.14.3313
Clinical features and management of primary biliary cirrhosis
Andrea Crosignani, Pier Maria Battezzati, Pietro Invernizzi, Carlo Selmi, Elena Prina, Mauro Podda
Andrea Crosignani, Pier Maria Battezzati, Pietro Invernizzi, Carlo Selmi, Elena Prina, Mauro Podda, Division of Internal Medicine and Liver Unit, San Paolo Hospital School of Medicine, University of Milan, Milano 20142, Italy
Pietro Invernizzi, Carlo Selmi, Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis, Davis, CA 95616, United States
Correspondence to: Andrea Crosignani, MD, Dipartimento di Medicina Interna, Polo Universitario, Ospedale San Paolo, Via di Rudinì 8, Milano 20142, Italy.
Telephone: +39-2-50323088
Fax: +39-2-50323089
Received: January 3, 2008
Revised: March 31, 2008
Accepted: April 7, 2008
Published online: June 7, 2008

Primary biliary cirrhosis (PBC), which is characterized by progressive destruction of intrahepatic bile ducts, is not a rare disease since both prevalence and incidence are increasing during the last years mainly due to the improvement of case finding strategies. The prognosis of the disease has improved due to both the recognition of earlier and indolent cases, and to the wide use of ursodeoxycholic acid (UDCA). New indicators of prognosis are available that will be useful especially for the growing number of patients with less severe disease. Most patients are asymptomatic at presentation. Pruritus may represent the most distressing symptom and, when UDCA is ineffective, cholestyramine represents the mainstay of treatment. Complications of long-standing cholestasis may be clinically relevant only in very advanced stages. Available data on the effects of UDCA on clinically relevant end points clearly indicate that the drug is able to slow but not to halt the progression of the disease while, in advanced stages, the only therapeutic option remains liver transplantation.

Keywords: Primary biliary cirrhosis, Epidemiology, Clinical course, Natural history, Treatment