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 language | English (US) |
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Pages (from-to) | 298-310 |
Number of pages | 13 |
Journal | Seminars in Liver Disease |
Volume | 25 |
Issue number | 3 |
DOIs | |
State | Published - Aug 2005 |
Externally published | Yes |
Keywords
- Anti-p62 nucleoporin antibodies
- Autoimmunity
- Nuclear antigens
- Nuclear dots
- Nuclear pore complexes
- Prognosis
- Rim-like
ASJC Scopus subject areas
- Hepatology