Overlap syndromes with autoimmune hepatitis in chronic cholestatic liver diseases.

Marina G. Silveira, Keith D. Lindor

Research output: Contribution to journalReview articlepeer-review

20 Scopus citations


Conditions exhibiting features of two different autoimmune liver diseases are commonly designated overlap syndromes, although there is no current agreement on what constitutes an overlap syndrome or specific diagnostic criteria. As in the classic autoimmune liver diseases, such as autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), the etiology is unknown but presumed to be related to alterations of immune regulation. Distinction of these clinical entities is important for management as outcomes may differ from outcomes of patients with diagnosis of classic autoimmune liver diseases. Due to their presumed rarity, no large therapeutic trials are available and treatment of overlap conditions is empirical and based upon extrapolation of data from the primary autoimmune liver diseases. PBC-AIH overlap is the most frequently described overlap syndrome and may be associated with a poor prognosis. This may represent an important and unrecognized cause of resistance to ursodeoxycholic acid in patients with PBC. PSC-AIH overlap is less commonly reported. Prognosis may be better than in patients with PSC alone; however, worse than in patients with AIH alone. Further studies are needed for determining diagnosis, natural history and optimal therapeutic strategies of overlap syndromes of autoimmune liver disease.

Original languageEnglish (US)
Pages (from-to)329-340
Number of pages12
JournalExpert review of gastroenterology & hepatology
Issue number2
StatePublished - Dec 2007
Externally publishedYes

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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