Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis
Open accessAbstract
Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti–smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(+) patients (26% vs. 5%, P = .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion , anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression. (Hepatology 2006;43:1135–1144.)
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PBC-specific ANA against gp210 and sp100 are seen more often in severe PBC and may predict an unfavorable course independent of serum bilirubin.
“PBC-specific ANA (antibodies against gp210 and sp100 antigens) are more frequently observed in patients with severe PBC and their presence may also be predictive of an unfavourable course, irrespective of serum bilirubin [75][76][77]63,78].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [77]
The Discussion (page unavailable) identifies gp210 and Sp100 as PBC-specific ANA targets and says PBC-specific ANA correlate with disease severity in cross-sectional studies. The Results (page unavailable) link anti-NPC antibodies, including anti-gp210, to poorer outcomes, but do not establish that anti-Sp100 predicts outcome. Anti-NPC added independent prognostic information only in patients with baseline bilirubin below 1 mg/dL, so the claim is too broad about prediction irrespective of bilirubin. (LLM, conf=0.88)
Experimental — accuracy not yet measuredPBC-specific ANA against gp210 and sp100 are more frequently observed in severe PBC and may predict an unfavourable course irrespective of serum bilirubin.
“PBC-specific ANA (antibodies against gp210 and sp100 antigens) are more frequently observed in patients with severe PBC and their presence may also be predictive of an unfavourable course, irrespective of serum bilirubin [75][76][77]63,78].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [77]
The Discussion identifies gp210 and Sp100 as PBC-specific ANA targets and describes an association between PBC-specific ANA and disease severity. The Results and Discussion link anti–nuclear pore complex antibodies, which include anti-gp210, to worse outcomes, particularly in patients with normal baseline bilirubin. They do not establish the same prognostic finding for anti-Sp100, and anti-NPC testing did not add independent prognostic value in the elevated-bilirubin group; thus, 'irrespective of serum bilirubin' is too broad. (LLM, conf=0.88)
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Self-citation
- Authors
- Wesierska-Gadek, J., Penner, E., Battezzati, P. M., Selmi, C., Zuin, M., Hitchman, E., Worman, H. J., Gershwin, E. M., Podda, M., & Invernizzi, P.
- Affiliations
- Medical University of Vienna (AT)
- University of Milan (IT)
- Columbia University (US)
- University of California, Davis (US)
- Corresponding author
- Invernizzi, Pietro
- Venue
- Hepatology
- Published
- 2006-05
- Volume
- 43
- Issue
- 5
- Pages
- 1135-1144
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W2086490001
- Semantic Scholar
- 4acfa8f4c231b124ebc878cbbec5a78d9bc4cc31
- Cited by
- 177
- References
- 43
- Citations by year
- 2026: 3 · 2025: 4 · 2024: 2 · 2023: 5 · 2022: 8 · 2021: 4 · 2020: 7 · 2019: 12 · 2018: 10 · 2017: 8 · 2016: 6 · 2015: 10 · 2014: 12 · 2013: 8 · 2012: 19
- FWCI
- 10.04
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (n01dk092310-000, DK 39588)
- National Institute of Diabetes and Digestive and Kidney Diseases (DK 39588, DK-92310)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- primary biliary cirrhosis, autoantibody profile, antimitochondrial antibodies, antinuclear antibodies, anti-smooth muscle antibodies, prognostic factors, disease progression, liver transplantation, survival, Mayo score, bilirubin
- MeSH terms
- Adult, Aged, Antibodies, Antinuclear, Autoantibodies, Female, Humans, Liver Cirrhosis, Biliary, Male, Middle Aged, Mitochondria, Prognosis, Prospective Studies, Biomarkers, Nuclear Pore
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