Characterization and clinical impact of antinuclear antibodies in primary biliary cirrhosis

Abstract

Antinuclear antibodies reactivities are present in more than half of primary biliary cirrhosis patients and target diverse autoantigens located in distinct subnuclear structures. Anti-gp210 identifies a subgroup of primary biliary cirrhosis patients with more serious liver disease. Positivity for anti-Sp100, anti-gp210, and anti-lamin B receptor, either alone or in combination, may act as a serologic marker of antimitochondrial antibodies negative primary biliary cirrhosis.

Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis

“…[22][23][24][25] However, this finding needs to be validated in larger longitudinal studies. Sp100 and gp210 proteins are the main antigenic targets of anti-ND and anti-nuclear pore complex antibodies, respectively.…”

Positive markers in AMA-negative PBC

“…The suggestion ensues that the clinicians using a panel of PBC-specific ANAs may have at their disposal a "positive" tool in the diagnosis of AMA-negative PBC. The findings of Muratori et al should be validated in larger series as the authors themselves appropriately advocate (12). The possibility that PBC-specific ANAs may become an essential marker for AMA-negative PBC is indirectly suggested by earlier immunofluorescence studies where the majority (6) or the totality (4) of such patients was found to be ANA positive.…”

Presence of disease specific autoantibodies against liver sinusoidal cells in primary biliary cirrhosis

“…Two PBC-specific ANA immunofluorescence patterns have been identified [11,12] : "multiple nuclear dots", corresponding to the antigens Sp100 and Sp140, promyelocytic leukemia (PML) nuclear body proteins and small ubiquitin-like modifiers (SUMOs) [13,14] , and "nuclear membrane" (rim), caused by anti-nuclear envelope antibodies (ANEAs), such as gp210 and nucleoporin p62 [15,16] . The anti-gp210 antibodies are highly specific for PBC and are associated with disease activity and severity [17,18] . Nakamura et al [19] found that the expression of gp210 is markedly increased on the nuclear envelope of small bile ducts and sometimes at infiltrating mononuclear cells in the portal area and/or periportal hepatocytes in PBC and this expression was positively correlated to disease activity.…”