ArticlePloS one2014
PR3-ANCA: a promising biomarker in primary sclerosing cholangitis (PSC).
Article in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 1 of them a synthesis that pooled it.
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Who cites it
36 citing papers in PubMed, 1 synthesis or guideline pooled it, 76 citations in OpenAlex.
- Guideline
- Mucosal Autoimmunity to Cell-Bound GP2 Isoforms Is a Sensitive Marker in PSC and Associated With the Clinical Phenotype.Frontiers in immunology · 2018Trial
- PSC-DM: A Validated Clinical Diagnostic Model for Distinguishing Primary From Secondary Sclerosing Cholangitis.Liver international : official journal of the International Association for the Study of the Liver · 2026Article
- Primary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges.Journal of clinical medicine · 2026Review
- Serum Anti-<italic>Saccharomyces cerevisiae</italic> Antibody Is Associated with Disease Severity in Patients with Primary Sclerosing Cholangitis.Digestive diseases (Basel, Switzerland) · 2026Article
- Antineutrophil Cytoplasmic Autoantibodies Specific to Bactericidal/Permeability-Increasing Protein: A Cross-Road Between Prolonged Gram-Negative Bacterial Infections and Ulcerative Colitis/Primary Sclerosing Cholangitis.Diagnostics (Basel, Switzerland) · 2025Article
- The impact of T cells on immune-related liver diseases: an overview.Inflammation and regeneration · 2025Review
- Management of IgG4-related cholangitis: diagnosis, therapy, and long-term surveillance.Gastroenterology report · 2025Review
- Primary sclerosing cholangitis: a narrative review of diagnostic and prognostic biomarkers.Translational gastroenterology and hepatology · 2025Review
- Serum syndecan1 has the potential to reflect activity at diagnosis and predict death during follow-up in patients with ANCA-associated vasculitis.Arthritis research & therapy · 2024Article
- Tubulin beta 5 is not the target of antineutrophil antibodies in primary sclerosing cholangitis.Clinical and experimental immunology · 2024Article
- Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD): a condition exemplifying the crosstalk of the gut-liver axis.Experimental & molecular medicine · 2023Review
- Exosomal Long Noncoding RNAs Serve as Biomarkers for Liver Disease.The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology · 2023Review
- PR3-ANCAs Detected by Third-Generation ELISA Predicts Severe Disease and Poor Survival in Primary Sclerosing Cholangitis.Diagnostics (Basel, Switzerland) · 2022Article
- Primary Sclerosing Cholangitis and Inflammatory Bowel Disease: A Review.Journal of clinical and translational hepatology · 2022Review
- Serological biomarkers for management of primary sclerosing cholangitis.World journal of gastroenterology · 2022Review
- A Review on Biomarkers for the Evaluation of Autoimmune Cholestatic Liver Diseases and Their Overlap Syndromes.Frontiers in molecular medicine · 2022Review
- Hepatic sarcoidosis resembling primary sclerosing cholangitis.BMJ case reports · 2021Article
- Anti-proteinase 3 antineutrophil cytoplasmic antibody reflects disease activity and predicts the response to steroid therapy in ulcerative colitis.BMC gastroenterology · 2021Article
- Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population-Single Center Study.Medicina (Kaunas, Lithuania) · 2021Article
Corrections and comments
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Authors and funding
13 authors at 7 institutions in 5 countries.
Funding
Abstract
BACKGROUND AND
aimsThe only recognized biomarker for primary sclerosing cholangitis (PSC) is atypical anti-neutrophil cytoplasmic antibodies (aANCA), which, in addition to having low sensitivity and specificity, is an indirect immunofluorescence (IIF) test lacking the advantages of high throughput and objectivity. Recent reports have shown that antibodies to proteinase-3 (PR3-ANCA) might add diagnostic value in inflammatory bowel disease (IBD), specifically in ulcerative colitis (UC). As PSC is associated with IBD, the objective of this study was to evaluate the frequency and clinical significance of PR3-ANCA in a large cohort of patients.
methodsA total of 244 PSC and 254 control [autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), hepatitis C viral infection (HCV), hepatitis B viral infection (HBV), and healthy controls] sera and their clinical correlations were retrospectively analyzed for PR3-ANCA determined by ELISA and a new chemiluminescence immunoassay (CIA). Testing was also performed for aANCA by IIF.
resultsWhen measured by CIA, PR3-ANCA was detected in 38.5% (94/244) of PSC patients compared to 10.6% (27/254) controls (p<0.0001). By ELISA, PR3-ANCA was detected in 23.4% (57/244) of PSC patients compared to 2.7% (6/254) controls (p<0.0001). PR3-ANCA in PSC patients was not associated with the presence or type of underlying IBD, and, in fact, it was more frequent in Crohn's disease (CD) patients with PSC than previously reported in CD alone. PR3-ANCA in PSC measured by CIA correlated with higher liver enzymes.
conclusionPR3-ANCA is detected in a significant proportion of PSC patients compared to other liver diseases including PBC and AIH. PR3-ANCA is associated with higher liver enzyme levels in PSC, and is not solely related to underlying IBD.
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Registered trials
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