Antinuclear antibodies in primary biliary cirrhosis.
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.
Invernizzi
Pietro
P
Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine, University of Milan, Italy. pietro.invernizzi@unimi.it
Selmi
Carlo
C
Ranftler
Carmen
C
Podda
Mauro
M
Wesierska-Gadek
Józefa
J
D000974:Antibodies, Antinuclear / Q000276:immunology*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000276:immunology* / Q000503:physiopathology; D009687:Nuclear Proteins / Q000276:immunology; D011379:Prognosis; D012720:Severity of Illness Index
D000974:Antibodies, Antinuclear; D009687:Nuclear Proteins