Antinuclear antibodies in primary biliary cirrhosis

Pietro Invernizzi, Carlo Selmi, Carmen Ranftler, Mauro Podda, Józefa Wesierska-Gadek

Research output: Contribution to journalArticle

137 Scopus citations

Abstract

Patients with primary biliary cirrhosis (PBC) generate a variety of autoantibodies, which are primarily directed against mitochondrial antigens (AMA). However, a subgroup of patient sera are also positive for antibodies to nuclear components (ANAs). At indirect immunofluorescence (UF), PBC sera mostly produce homogeneous, nuclear dot, speckled, centromere, or rim-like patterns. During the last two decades, a number of nuclear structures have been recognized as specific targets of ANA in PBC. These include Sp100 and promyelocytic leukemia proteins, which generate a nuclear dot UF pattern, and two components of the nuclear pore complex specifically associated with a perinuclear pattern (i.e., gp210 and p62). In recent years, the clinical significance of ANA in PBC has been widely investigated and data indicate that, unlike AMAs, PBC-specific ANAs correlate with disease severity and may therefore be a marker of poor prognosis.

Original languageEnglish (US)
Pages (from-to)298-310
Number of pages13
JournalSeminars in Liver Disease
Volume25
Issue number3
DOIs
StatePublished - Aug 2005
Externally publishedYes

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Keywords

  • Anti-p62 nucleoporin antibodies
  • Autoimmunity
  • Nuclear antigens
  • Nuclear dots
  • Nuclear pore complexes
  • Prognosis
  • Rim-like

ASJC Scopus subject areas

  • Hepatology

Cite this

Invernizzi, P., Selmi, C., Ranftler, C., Podda, M., & Wesierska-Gadek, J. (2005). Antinuclear antibodies in primary biliary cirrhosis. Seminars in Liver Disease, 25(3), 298-310. https://doi.org/10.1055/s-2005-916321