Clinical features and management of primary biliary cirrhosis

Andrea Crosignani, Pier Maria Battezzati, Pietro Invernizzi, Carlo Selmi, Elena Prina, Mauro Podda

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31 Scopus citations

Abstract

Primary biliary cirrhosis (PBQ, which is characterised 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.

Original languageEnglish (US)
Pages (from-to)3313-3327
Number of pages15
JournalWorld Journal of Gastroenterology
Volume14
Issue number21
DOIs
StatePublished - 2008

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Keywords

  • Clinical course
  • Epidemiology
  • Natural history
  • Primary biliary cirrhosis
  • Treatment

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Crosignani, A., Battezzati, P. M., Invernizzi, P., Selmi, C., Prina, E., & Podda, M. (2008). Clinical features and management of primary biliary cirrhosis. World Journal of Gastroenterology, 14(21), 3313-3327. https://doi.org/10.3748/wjg.14.3313