ArticleLiver international : official journal of the International Association for the Study of the Liver2014
Small duct primary sclerosing cholangitis without inflammatory bowel disease is genetically different from large duct disease.
Article in Liver international : official journal of the International Association for the Study of the Liver, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 53 citations in OpenAlex.
- Univariate- and machine learning-based plasma metabolite signature differentiates PSC-IBD from IBD and is predicted to be driven by gut microbial changes.Metabolomics : Official journal of the Metabolomic Society · 2026Article
- Correspondence to editorial on "Safety and efficacy of HK-660S in patients with primary sclerosing cholangitis: A randomized double-blind phase 2a trial".Clinical and molecular hepatology · 2025Article
- Challenges and innovations in primary sclerosing cholangitis clinical trials: Evaluating HK-660S and the path forward: Editorial on "Safety and efficacy of HK-660S in patients with primary sclerosing cholangitis: A randomized double-blind phase 2a trial".Clinical and molecular hepatology · 2025Article
- Morphological aspects of small-duct cholangiopathies: A minireview.World journal of hepatology · 2023Review
- Primary sclerosing cholangitis: review for radiologists.Abdominal radiology (New York) · 2023Review
- Small duct primary sclerosing cholangitis: A discrete variant or a bridge to large duct disease, a practical review.World journal of hepatology · 2022Review
- Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population-Single Center Study.Medicina (Kaunas, Lithuania) · 2021Article
- Role of Endoscopy in Primary Sclerosing Cholangitis.Clinical endoscopy · 2021Review
- HLA haplotypes in primary sclerosing cholangitis patients of admixed and non-European ancestry.HLA · 2017Article
- Variations in primary sclerosing cholangitis across the age spectrum.Journal of gastroenterology and hepatology · 2017Article
- Primary Sclerosing Cholangitis.The New England journal of medicine · 2016Review
- Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?Inflammatory intestinal diseases · 2016Review
- Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.Clinics in liver disease · 2016Review
- The Immunogenetics of Autoimmune Cholestasis.Clinics in liver disease · 2016Review
- A Review of Extraintestinal Manifestations and Complications of Inflammatory Bowel Disease.Saudi journal of medicine & medical sciencesReview
Corrections and comments
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Authors and funding
15 authors at 7 institutions in 6 countries.
Funding
Abstract
BACKGROUND &
aimsSmall duct primary sclerosing cholangitis (PSC) is phenotypically a mild version of large duct PSC, but it is unknown whether these phenotypes share aetiology. We aimed to characterize their relationship by investigating genetic associations in the human leucocyte antigen (HLA) complex, which represent the strongest genetic risk factors in large duct PSC.
methodsFour classical HLA loci (HLA-A, HLA-B, HLA-C and HLA-DRB1) were genotyped in 87 small duct PSC patients, 485 large duct PSC patients and 1117 controls across three geographical regions.
resultsHLA-DRB1*13:01 (OR = 2.0, 95% CI 1.2-3.4, P = 0.01) and HLA-B*08 (OR = 1.6, 95% CI 1.1-2.4, P = 0.02) were significantly associated with small duct PSC compared with healthy controls. Based on the observed frequency of HLA-B*08 in small duct PSC, the strongest risk factor in large duct PSC, an estimated 32% (95% CI 4-65%) of this population can be hypothesized to represent early stages or mild variants of large duct PSC. This subgroup may be constituted by small duct PSC patients with inflammatory bowel disease (IBD), which greatly resembled large duct PSC in its HLA association. In contrast, small duct PSC without IBD was only associated with HLA-DRB1*13:01(P = 0.03) and was otherwise distinctly dissimilar from large duct PSC.
conclusionsSmall duct PSC with IBD resembles large duct PSC in its HLA association and may represent early stages or mild variants of large duct disease. Different HLA associations in small duct PSC without IBD could indicate that this subgroup is a different entity. HLA-DRB1*13:01 may represent a specific risk factor for inflammatory bile duct disease.
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Registered trials
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