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. Copyright (copyright) 2005 by Thieme Medical Publishers, Inc.

Antinuclear antibodies in primary biliary cirrhosis / P. Invernizzi, C. Selmi, C. Ranftler, M. Podda, J. Wesierska-Gadek. - In: SEMINARS IN LIVER DISEASE. - ISSN 0272-8087. - 25:3(2005 Aug), pp. 298-310.

Antinuclear antibodies in primary biliary cirrhosis

P. Invernizzi;C. Selmi;M. Podda;
2005

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. Copyright (copyright) 2005 by Thieme Medical Publishers, Inc.
Anti-p62 nucleoporin antibodies; Autoimmunity; Nuclear antigens; Nuclear dots; Nuclear pore complexes; Prognosis; Rim-like
Settore MED/09 - Medicina Interna
ago-2005
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/64900
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