gp210 is a 209 kDa transmembrane glycoprotein located in the nuclear pore complex . Anti-gp210 IgG antibodies are disease-specific antinuclear antibodies (ANAs) that serve as biomarkers for PBC, particularly in patients negative for antimitochondrial antibodies (AMA) . These antibodies are highly specific (>98%) but exhibit low sensitivity (~25%) for PBC .
AMA-Negative PBC: Anti-gp210 antibodies aid in diagnosing PBC in ~30% of AMA-negative patients .
Diagnostic Criteria: The presence of anti-gp210 antibodies, alongside elevated alkaline phosphatase (ALP) and histologic evidence, confirms PBC .
Anti-gp210 IgG Level (Units) | Interpretation |
---|---|
≤20.1 | Negative |
20.1–24.9 | Equivocal |
≥25.0 | Positive |
Anti-gp210 positivity correlates with aggressive disease progression and poor outcomes:
Mortality: 2.38-fold increased risk of death or liver transplantation .
Treatment Response: 39.3% of anti-gp210-positive patients fail ursodeoxycholic acid (UDCA) therapy vs. 16.7% in negative cohorts .
Parameter | Anti-gp210 (+) | Anti-gp210 (−) |
---|---|---|
5-Year Transplant-Free Survival | 77.0% | 90.3% |
Liver-Related Mortality | 22.0% | 9.2% |
Histologic Severity* | Higher | Lower |
*Includes lobular inflammation, interfacial hepatitis, and bile duct injury .
Pathogenesis: Molecular mimicry triggered by bacterial or endogenous peptides may drive anti-gp210 production .
Immune Response: T cells and cytokines (e.g., IL-12, IFN-γ) contribute to gp210 antigen presentation and antibody generation .
Anti-gp210 antibodies are detected via semi-quantitative ELISA, with results reported in 1–8 days . Notably, HEp-2 indirect immunofluorescence (IFA) is unreliable for gp210 detection, necessitating confirmatory immunoassays .