Semin Liver Dis 2005; 25(3): 298-310
DOI: 10.1055/s-2005-916321
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Antinuclear Antibodies in Primary Biliary Cirrhosis

Pietro Invernizzi1 , 2 , Carlo Selmi1 , Carmen Ranftler3 , Mauro Podda2 , Józefa Wesierska-Gadek3
  • 1Professor, Division of Internal Medicine, Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
  • 2Division of Internal Medicine, Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy
  • 3Cell Cycle Regulation Group, Division: Institute of Cancer Research, Department of Internal Medicine I, Vienna Medical University, Vienna, Austria
Further Information

Publication History

Publication Date:
06 September 2005 (online)

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 (IIF), 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 IIF 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.

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Pietro InvernizziM.D. Ph.D. 

Division of Internal Medicine, Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine

University of Milan, Via di Rudinì 8

20142 Milano, Italy

Email: pietro.invernizzi@unimi.it

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