Antimitochondrial Antibody–Negative Primary Biliary Cholangitis: Is It Really the Same Disease?

David M. Chascsa, Keith Lindor

Research output: Contribution to journalReview articlepeer-review

13 Scopus citations


Antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC) is a term reserved for patients with clinical and histopathological findings consistent with PBC but without positive AMA. There does not seem to be a natural progression from AMA negativity to positivity. Antinuclear and antismooth muscle antibodies are frequently found in the absence of histologic autoimmune hepatitis features. The disease course may be more severe than AMA-positive. Response to standard therapy for PBC and autoimmune hepatitis varies. Nevertheless, there is insufficient evidence to suggest AMA-negative PBC is different enough to warrant classification as a separate disease from AMA-positive PBC.

Original languageEnglish (US)
Pages (from-to)589-601
Number of pages13
JournalClinics in liver disease
Issue number3
StatePublished - Aug 2018


  • Antimitochondrial antibody (AMA)
  • Antinuclear antibody (ANA)
  • Primary biliary cholangitis (PBC)
  • Ursodeoxycholic acid (UDCA)

ASJC Scopus subject areas

  • Hepatology


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