The DOI matches its Crossref record; 155 of the 155 references it cites were found in a registry or an index.
Primary sclerosing cholangitis
Open accessAbstract
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography. Genetic and environmental factors are important in the cause of the disease, with the intestinal microbiome increasingly thought to play a pathogenetic role. Approximately 70% of patients have concurrent inflammatory bowel disease and patients require colonoscopic screening and surveillance. Primary sclerosing cholangitis is associated with increased malignancy risk and surveillance strategies for early cholangiocarcinoma detection are limited. No single drug has been proven to improve transplant-free survival. Liver transplantation is effective for advanced disease but at least 25% of patients develop recurrent disease in the graft.
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1 retracted
Doorenspleet, M. E., Hubers, L. M., Culver, E. L., Wenniger, L. J. M. D. B., Klarenbeek, P. L., Chapman, R. W., Baas, F., van de Graaf, S. F. J., Verheij, J., van Gulik, T. M., et al. (2016) · Hepatology · doi:10.1002/hep.28568 · Cited by 97
Self-citationRetractedA Model to Predict Survival in Patients With End–Stage Liver Disease
Kamath, P. S., Wiesner, R. H., Malinchoc, M., Kremers, W., Therneau, T. M., Kosberg, C. L., D'Amico, G., Dickson, R. E., & Kim, R. W. (2001) · Hepatology · doi:10.1053/jhep.2001.22172 · Cited by 4,975
Alvarez, F., Berg, P. A., Bianchi, F. B., Bianchi, L., Burroughs, A. K., Cancado, E. L., Chapman, R. W., Cooksley, W. G. E., Czaja, A. J., Desmet, V. J., et al. (1999) · Journal of Hepatology · doi:10.1016/s0168-8278(99)80297-9 · Cited by 2,903
High Serum IgG4 Concentrations in Patients with Sclerosing Pancreatitis
Hamano, H., Kawa, S., Horiuchi, A., Unno, H., Furuya, N., Akamatsu, T., Fukushima, M., Nikaido, T., Nakayama, K., Usuda, N., et al. (2001) · New England Journal of Medicine · doi:10.1056/nejm200103083441005 · Cited by 2,705
Stone, J. H., Zen, Y., & Deshpande, V. (2012) · New England Journal of Medicine · doi:10.1056/nejmra1104650 · Cited by 2,616
Simplified criteria for the diagnosis of autoimmune hepatitis
Hennes, E. M., Zeniya, M., Czaja, A. J., Parés, A., Dalekos, G. N., Krawitt, E. L., Bittencourt, P. L., Porta, G., Boberg, K. M., Hofer, H., et al. (2008) · Hepatology · doi:10.1002/hep.22322 · Cited by 1,901
Self-citationEASL Clinical Practice Guidelines: management of cholestatic liver diseases
European Association for the Study of the Liver (2009) · Journal of Hepatology · doi:10.1016/j.jhep.2009.04.009 · Cited by 1,729
Diagnosis and Management of Primary Sclerosing Cholangitis
Chapman, R., Fevery, J., Kalloo, A., Nagorney, D. M., Boberg, K. M., Shneider, B., & Gores, G. J. (2010) · Hepatology · doi:10.1002/hep.23294 · Cited by 1,224
Cairns, S. R., Scholefield, J. H., Steele, R. J., Dunlop, M. G., Thomas, H. J. W., Evans, G. D., Eaden, J. A., Rutter, M. D., Atkin, W. P., Saunders, B. P., et al. (2010) · Gut · doi:10.1136/gut.2009.179804 · Cited by 1,141
Primary sclerosing cholangitis – a comprehensive review
Karlsen, T. H., Folseraas, T., Thorburn, D., & Vesterhus, M. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.07.022 · Cited by 868
Cited by
Nakamoto, N., Sasaki, N., Aoki, R., Miyamoto, K., Suda, W., Teratani, T., Suzuki, T., Koda, Y., Chu, P.-S., Taniki, N., et al. (2019) · Nature Microbiology · doi:10.1038/s41564-018-0333-1 · Cited by 425
EASL Clinical Practice Guidelines on sclerosing cholangitis
Chazouilleres, O., Beuers, U., Bergquist, A., Karlsen, T. H., Levy, C., Samyn, M., Schramm, C., & Trauner, M. (2022) · Journal of Hepatology · doi:10.1016/j.jhep.2022.05.011 · Cited by 419
Self-citationde Vries, E., Bolier, R., Goet, J., Parés, A., Verbeek, J., de Vree, M., Drenth, J., van Erpecum, K., van Nieuwkerk, K., van der Heide, F., et al. (2021) · Gastroenterology · doi:10.1053/j.gastro.2020.10.001 · Cited by 224
Self-citationAlterations of the bile microbiome in primary sclerosing cholangitis
Liwinski, T., Zenouzi, R., John, C., Ehlken, H., Rühlemann, M. C., Bang, C., Groth, S., Lieb, W., Kantowski, M., Andersen, N., et al. (2020) · Gut · doi:10.1136/gutjnl-2019-318416 · Cited by 149
Self-citationEpithelial Plasticity during Liver Injury and Regeneration
Gadd, V. L., Aleksieva, N., & Forbes, S. J. (2020) · Cell Stem Cell · doi:10.1016/j.stem.2020.08.016 · Cited by 142
Metadata
- Authors
- Dyson, J. K., Beuers, U., Jones, D. E. J., Lohse, A. W., & Hudson, M.
- Venue
- The Lancet
- Publisher
- Elsevier BV
- Published
- 2018-06
- Volume
- 391
- Issue
- 10139
- Pages
- 2547-2559
- ISSN
- 0099-5355 (ISSN-L)
- 0140-6736
- OpenAlex
- W2785876103
- Cited by
- 462
- References
- 155
- FWCI
- 18.00
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Primary sclerosing cholangitis. https://app.cochat.ai/papers/10.1016/s0140-6736(18)30300-3
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- Dyson, J. K., Beuers, U., Jones, D. E. J., Lohse, A. W., & Hudson, M. (2018). Primary sclerosing cholangitis. The Lancet. https://doi.org/10.1016/s0140-6736(18)30300-3
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
