Antimitochondrial Antibody–Negative Primary Biliary Cholangitis

Is It Really the Same Disease?

David M. Chascsa, Keith Lindor

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

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)
JournalClinics in Liver Disease
DOIs
StateAccepted/In press - Jan 1 2018

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Cholangitis
Antibodies
Autoimmune Hepatitis
Muscles

Keywords

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

ASJC Scopus subject areas

  • Hepatology

Cite this

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title = "Antimitochondrial Antibody–Negative Primary Biliary Cholangitis: Is It Really the Same Disease?",
abstract = "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.",
keywords = "Antimitochondrial antibody (AMA), Antinuclear antibody (ANA), Primary biliary cholangitis (PBC), Ursodeoxycholic acid (UDCA)",
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AU - Lindor, Keith

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N2 - 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.

AB - 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.

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KW - Antinuclear antibody (ANA)

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KW - Ursodeoxycholic acid (UDCA)

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