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Most patients eventually require liver transplantation or develop serious complications, the most severe being end-stage liver disease and cholangiocarcinoma. The global prevalence of PSC is rising and has been reported to reach up to 31.7 cases per 100,000 individuals, representing a significant challenge in both diagnosis and management. In this review, we aim to provide a clinically oriented overview of the diagnosis and management of PSC. Furthermore, we seek to highlight key challenges and areas of uncertainty that clinicians encounter in the diagnosis and treatment of patients with this complex disease.<\/jats:p>","DOI":"10.3390\/jcm15031149","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T09:48:08Z","timestamp":1770025688000},"page":"1149","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Primary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges"],"prefix":"10.3390","volume":"15","author":[{"given":"Sofia","family":"Svensson Di Giorgio","sequence":"first","affiliation":[{"name":"Department of Diagnostics and Intervention, Surgery, Ume\u00e5 University, 901 87 Ume\u00e5, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara Maria","family":"Scandavini","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Intervention, Surgery, Ume\u00e5 University, 901 87 Ume\u00e5, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Molinaro","sequence":"additional","affiliation":[{"name":"Department of Molecular and Clinical Medicine, Wallenberg Laboratory, University of Gothenburg, 405 30 Gothenburg, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1843-5673","authenticated-orcid":false,"given":"Urban","family":"Arnelo","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Intervention, Surgery, Ume\u00e5 University, 901 87 Ume\u00e5, Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6785-2326","authenticated-orcid":false,"given":"Roberto","family":"Valente","sequence":"additional","affiliation":[{"name":"Department of Diagnostics and Intervention, Surgery, Ume\u00e5 University, 901 87 Ume\u00e5, Sweden"},{"name":"Department of Surgery, Division of Surgical Oncology, University of Colorado School of Medicine, Aurora, CO 80045, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1056\/NEJMra1506330","article-title":"Primary Sclerosing Cholangitis","volume":"375","author":"Lazaridis","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1056\/NEJM199504063321406","article-title":"Primary sclerosing cholangitis","volume":"332","author":"Lee","year":"1995","journal-title":"N. Engl. J. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1764","DOI":"10.1053\/j.gastro.2021.07.046","article-title":"Defining Primary Sclerosing Cholangitis: Results from an International Primary Sclerosing Cholangitis Study Group Consensus Process","volume":"161","author":"Ponsioen","year":"2021","journal-title":"Gastroenterology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1016\/j.jhep.2017.07.022","article-title":"Primary sclerosing cholangitis\u2014A comprehensive review","volume":"67","author":"Karlsen","year":"2017","journal-title":"J. Hepatol."},{"key":"ref_5","first-page":"2418","article-title":"Global incidence, prevalence and features of primary sclerosing cholangitis: A systematic review and meta-analysis","volume":"41","author":"Mehta","year":"2021","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_6","first-page":"802","article-title":"Immune-mediated diseases in primary sclerosing cholangitis","volume":"43","author":"Lamberts","year":"2011","journal-title":"Dig. Liver Dis. Off. J. Ital. Soc. Gastroenterol. Ital. Assoc. Study Liver"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"3195","DOI":"10.1111\/j.1572-0241.2000.03292.x","article-title":"Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis","volume":"95","author":"Saarinen","year":"2000","journal-title":"Am. J. Gastroenterol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1865","DOI":"10.1053\/j.gastro.2021.08.032","article-title":"Prevalence of Primary Sclerosing Cholangitis in Patients with Inflammatory Bowel Disease: A Systematic Review and Meta-analysis","volume":"161","author":"Barberio","year":"2021","journal-title":"Gastroenterology"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2045","DOI":"10.1002\/hep.26565","article-title":"Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis","volume":"58","author":"Boonstra","year":"2013","journal-title":"Hepatology"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1080\/00365521.2019.1707277","article-title":"Epidemiology, risk of malignancy and patient survival in primary sclerosing cholangitis: A population-based study in Finland","volume":"55","author":"Pukkala","year":"2020","journal-title":"Scand. J. Gastroenterol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1097\/MOG.0000000000000335","article-title":"Risk of cholangiocarcinoma in patients with primary sclerosing cholangitis: Diagnosis and surveillance","volume":"33","author":"Ehlken","year":"2017","journal-title":"Curr. Opin. Gastroenterol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1097\/MOG.0000000000000333","article-title":"The evolution of natural history of primary sclerosing cholangitis","volume":"33","author":"Takakura","year":"2017","journal-title":"Curr. Opin. Gastroenterol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3123","DOI":"10.1007\/s10620-006-9681-4","article-title":"Incidence, diagnosis, and therapy of cholangiocarcinoma in patients with primary sclerosing cholangitis","volume":"52","author":"Fevery","year":"2007","journal-title":"Dig. Dis. Sci."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1053\/j.gastro.2020.05.049","article-title":"Effects of Primary Sclerosing Cholangitis on Risks of Cancer and Death in People with Inflammatory Bowel Disease, Based on Sex, Race, and Age","volume":"159","author":"Trivedi","year":"2020","journal-title":"Gastroenterology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/S0168-8278(01)00288-4","article-title":"Hepatic and extrahepatic malignancies in primary sclerosing cholangitis","volume":"36","author":"Bergquist","year":"2002","journal-title":"J. Hepatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1975","DOI":"10.1053\/j.gastro.2017.02.038","article-title":"Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate with Course of Primary Sclerosing Cholangitis","volume":"152","author":"Trivedi","year":"2017","journal-title":"Gastroenterology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/0027-5107(75)90115-3","article-title":"Proceedings: Structure-activity considerations of alkylating agents as mutagens","volume":"29","author":"Bedford","year":"1975","journal-title":"Mutat. Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1111\/j.1572-0241.2004.04067.x","article-title":"Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis","volume":"99","author":"Burak","year":"2004","journal-title":"Am. J. Gastroenterol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/j.jhep.2008.08.013","article-title":"High lifetime risk of cancer in primary sclerosing cholangitis","volume":"50","author":"Claessen","year":"2009","journal-title":"J. Hepatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e0442","DOI":"10.1097\/HC9.0000000000000442","article-title":"Surveillance MRI is associated with improved survival in patients with primary sclerosing cholangitis","volume":"8","author":"Tan","year":"2024","journal-title":"Hepatol. Commun."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1038\/ajg.2015.112","article-title":"American College of Gastroenterology ACG Clinical Guideline: Primary Sclerosing Cholangitis","volume":"110","author":"Lindor","year":"2015","journal-title":"Am. J. Gastroenterol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.1136\/gutjnl-2018-317993","article-title":"British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis","volume":"68","author":"Chapman","year":"2019","journal-title":"Gut"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1038\/s41572-025-00600-x","article-title":"Primary sclerosing cholangitis","volume":"11","author":"Manns","year":"2025","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1111\/j.1477-2574.2010.00268.x","article-title":"Analytical review of diagnosis and treatment strategies for dominant bile duct strictures in patients with primary sclerosing cholangitis","volume":"13","author":"Aljiffry","year":"2011","journal-title":"HPB"},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"European Association for the Study of the Liver EASL (2009). Clinical Practice Guidelines: Management of cholestatic liver diseases. J. Hepatol., 51, 237\u2013267.","DOI":"10.1016\/j.jhep.2009.04.009"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"European Association for the Study of the Liver EASL (2022). Clinical Practice Guidelines on sclerosing cholangitis. J. Hepatol., 77, 761\u2013806.","DOI":"10.1016\/j.jhep.2022.05.011"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1111\/j.1572-0241.2006.00872.x","article-title":"Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study","volume":"102","author":"Tischendorf","year":"2007","journal-title":"Am. J. Gastroenterol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1053\/jhep.2002.31872","article-title":"Time-dependent Cox regression model is superior in prediction of prognosis in primary sclerosing cholangitis","volume":"35","author":"Boberg","year":"2002","journal-title":"Hepatology"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1016\/S0025-6196(11)64614-4","article-title":"A revised natural history model for primary sclerosing cholangitis","volume":"75","author":"Kim","year":"2000","journal-title":"Mayo Clin. Proc."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1136\/gut.21.10.870","article-title":"Primary sclerosing cholangitis: A review of its clinical features, cholangiography, and hepatic histology","volume":"21","author":"Chapman","year":"1980","journal-title":"Gut"},{"key":"ref_31","unstructured":"Rawla, P., and Samant, H. (2025, February 10). Primary Sclerosing Cholangitis, StatPearls, Available online: http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK537181\/."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Stinton, L.M., Bentow, C., Mahler, M., Norman, G.L., Eksteen, B., Mason, A.L., Kaplan, G.G., Lindkvist, B., Hirschfield, G.M., and Milkiewicz, P. (2014). PR3-ANCA: A promising biomarker in primary sclerosing cholangitis (PSC). PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0112877"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1016\/S0168-8278(00)80241-X","article-title":"Clinical significance of anti-neutrophil cytoplasmic antibodies (ANCA) in autoimmune liver diseases","volume":"32","author":"Roozendaal","year":"2000","journal-title":"J. Hepatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1385","DOI":"10.1016\/0016-5085(91)70028-V","article-title":"Neutrophil cytoplasmic antibodies: A link between primary sclerosing cholangitis and ulcerative colitis","volume":"100","author":"Duerr","year":"1991","journal-title":"Gastroenterology"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.clinre.2012.07.008","article-title":"Antineutrophil cytoplasmic auto-antibodies (ANCA) in autoimmune hepatitis and primary sclerosing cholangitis","volume":"37","author":"Deniziaut","year":"2013","journal-title":"Clin. Res. Hepatol. Gastroenterol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S0002-9343(98)00294-0","article-title":"Antineutrophil cytoplasmic antibodies in primary sclerosing cholangitis: Defined specificities may be associated with distinct clinical features","volume":"105","author":"Roozendaal","year":"1998","journal-title":"Am. J. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1080\/08916930400022673","article-title":"Antineutrophil cytoplasmic antibodies (ANCA)","volume":"38","author":"Radice","year":"2005","journal-title":"Autoimmunity"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"102787","DOI":"10.1016\/j.autrev.2021.102787","article-title":"Anti-neutrophil cytoplasm antibodies (ANCA) in autoimmune diseases: A matter of laboratory technique and clinical setting","volume":"20","author":"Granito","year":"2021","journal-title":"Autoimmun. Rev."},{"key":"ref_39","first-page":"458","article-title":"Antineutrophil antibodies define clinical and genetic subgroups in primary sclerosing cholangitis","volume":"37","author":"Hov","year":"2017","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1111\/j.1365-2036.2005.02417.x","article-title":"Anti-Saccharomyces cerevisiae and perinuclear anti-neutrophil cytoplasmic antibodies in coeliac disease before and after gluten-free diet","volume":"21","author":"Granito","year":"2005","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/S0091-6749(05)80067-3","article-title":"A distinct subset of antineutrophil cytoplasmic antibodies is associated with inflammatory bowel disease","volume":"86","author":"Saxon","year":"1990","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2547","DOI":"10.1016\/S0140-6736(18)30300-3","article-title":"Primary sclerosing cholangitis","volume":"391","author":"Dyson","year":"2018","journal-title":"Lancet"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2291","DOI":"10.3748\/wjg.v28.i21.2291","article-title":"Serological biomarkers for management of primary sclerosing cholangitis","volume":"28","author":"Tornai","year":"2022","journal-title":"World J. Gastroenterol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1053\/bega.2001.0209","article-title":"Anti-neutrophil antibodies in primary sclerosing cholangitis","volume":"15","author":"Terjung","year":"2001","journal-title":"Best Pract. Res. Clin. Gastroenterol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1046\/j.1365-2249.2003.02166.x","article-title":"Anti-Saccharomyces cerevisiae antibodies (ASCA) and autoimmune liver diseases","volume":"132","author":"Muratori","year":"2003","journal-title":"Clin. Exp. Immunol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"100385","DOI":"10.1016\/j.jhepr.2021.100385","article-title":"Blue-collar work is a risk factor for developing IgG4-related disease of the biliary tract and pancreas","volume":"3","author":"Hubers","year":"2021","journal-title":"JHEP Rep. Innov. Hepatol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1453","DOI":"10.1002\/hep.26999","article-title":"Exposure to occupational antigens might predispose to IgG4-related disease","volume":"60","author":"Culver","year":"2014","journal-title":"Hepatology"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1053\/j.gastro.2007.12.009","article-title":"Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy","volume":"134","author":"Ghazale","year":"2008","journal-title":"Gastroenterology"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1002\/hep.26977","article-title":"Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis","volume":"59","author":"Boonstra","year":"2014","journal-title":"Hepatology"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1002\/hep.29204","article-title":"The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration","volume":"66","author":"Deneau","year":"2017","journal-title":"Hepatology"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1016\/S0168-8278(99)80297-9","article-title":"International Autoimmune Hepatitis Group Report: Review of criteria for diagnosis of autoimmune hepatitis","volume":"31","author":"Alvarez","year":"1999","journal-title":"J. Hepatol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1016\/S0168-8278(00)80005-7","article-title":"High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis","volume":"33","author":"Terkivatan","year":"2000","journal-title":"J. Hepatol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1002\/hep.32771","article-title":"AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma","volume":"77","author":"Bowlus","year":"2023","journal-title":"Hepatology"},{"key":"ref_54","first-page":"2329","article-title":"The role of magnetic resonance imaging and endoscopic retrograde cholangiography in the evaluation of disease activity and severity in primary sclerosing cholangitis","volume":"38","author":"Tenca","year":"2018","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"170","DOI":"10.32074\/1591-951X-245","article-title":"Autoimmune biliary diseases: Primary biliary cholangitis and primary sclerosing cholangitis","volume":"113","author":"Sarcognato","year":"2021","journal-title":"Pathologica"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1055\/s-2006-933559","article-title":"Etiopathogenesis of primary sclerosing cholangitis","volume":"26","author":"Vierling","year":"2006","journal-title":"Semin. Liver Dis."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1002\/hep.28363","article-title":"Toxic Bile and Sclerosing Cholangitis: Is there a role for pharmacological interruption of the bile acid enterohepatic circulation?","volume":"63","author":"Dawson","year":"2016","journal-title":"Hepatology"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1016\/j.jhep.2015.06.018","article-title":"Activation of biliary tree stem cells within peribiliary glands in primary sclerosing cholangitis","volume":"63","author":"Carpino","year":"2015","journal-title":"J. Hepatol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1873","DOI":"10.1136\/gutjnl-2017-314249","article-title":"Cholangiocytes and the environment in primary sclerosing cholangitis: Where is the link?","volume":"66","author":"Karlsen","year":"2017","journal-title":"Gut"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1002\/hep.1840140305","article-title":"Increased expression of intercellular adhesion molecule 1 on bile ducts in primary biliary cirrhosis and primary sclerosing cholangitis","volume":"14","author":"Adams","year":"1991","journal-title":"Hepatology"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/S0168-8278(96)80292-3","article-title":"Increased intercellular adhesion molecule-1 serum concentration in cholestasis","volume":"25","author":"Polzien","year":"1996","journal-title":"J. Hepatol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1016\/j.jhep.2021.03.029","article-title":"Downregulation of TGR5 (GPBAR1) in biliary epithelial cells contributes to the pathogenesis of sclerosing cholangitis","volume":"75","author":"Reich","year":"2021","journal-title":"J. Hepatol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"100561","DOI":"10.1016\/j.jhepr.2022.100561","article-title":"Suppression of bile acid synthesis as a tipping point in the disease course of primary sclerosing cholangitis","volume":"4","author":"Braadland","year":"2022","journal-title":"JHEP Rep. Innov. Hepatol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1038\/s42255-021-00452-1","article-title":"Gut microbiota depletion exacerbates cholestatic liver injury via loss of FXR signalling","volume":"3","author":"Schneider","year":"2021","journal-title":"Nat. Metab."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"187","DOI":"10.3109\/00365528209182038","article-title":"HLA antigens and immunoregulatory T cells in ulcerative colitis associated with hepatobiliary disease","volume":"17","author":"Schrumpf","year":"1982","journal-title":"Scand. J. Gastroenterol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1053\/j.gastro.2009.11.046","article-title":"Genome-wide association analysis in primary sclerosing cholangitis","volume":"138","author":"Karlsen","year":"2010","journal-title":"Gastroenterology"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1136\/gut.51.4.567","article-title":"Cigarette smoking, appendectomy, and tonsillectomy as risk factors for the development of primary sclerosing cholangitis: A case control study","volume":"51","author":"Mitchell","year":"2002","journal-title":"Gut"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.crad.2013.12.008","article-title":"Diagnostic workup of primary sclerosing cholangitis: The benefit of adding gadoxetic acid-enhanced T1-weighted magnetic resonance cholangiography to conventional T2-weighted magnetic resonance cholangiography","volume":"69","author":"Nolz","year":"2014","journal-title":"Clin. Radiol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1148\/radiol.10091953","article-title":"Primary sclerosing cholangitis: Meta-analysis of diagnostic performance of MR cholangiopancreatography","volume":"256","author":"Dave","year":"2010","journal-title":"Radiology"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"47","DOI":"10.3390\/tomography10010005","article-title":"Primary Sclerosing Cholangitis: Diagnostic Criteria","volume":"10","author":"Cazzagon","year":"2024","journal-title":"Tomography"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1055\/s-2008-1040417","article-title":"Small-duct primary sclerosing cholangitis","volume":"11","author":"Ludwig","year":"1991","journal-title":"Semin. Liver Dis."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"6495","DOI":"10.1007\/s00330-025-11583-4","article-title":"ESGAR consensus statement on MR imaging in primary sclerosing cholangitis","volume":"35","author":"Ippolito","year":"2025","journal-title":"Eur. Radiol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"VII","DOI":"10.1590\/0100-3984.2023.56.6e2","article-title":"Advancing prognostic assessment in primary sclerosing cholangitis: Insights from abdominal magnetic resonance imaging and magnetic resonance cholangiopancreatography with Anali scores","volume":"56","author":"Schuch","year":"2023","journal-title":"Radiol. Bras."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"100595","DOI":"10.1016\/j.jhepr.2022.100595","article-title":"Development of a prognostic MRCP-score (DiStrict) for individuals with large-duct primary sclerosing cholangitis","volume":"4","author":"Grigoriadis","year":"2022","journal-title":"JHEP Rep. Innov. Hepatol."},{"key":"ref_75","first-page":"e70348","article-title":"Radiomics-Based Prognostication in Primary Sclerosing Cholangitis: A Proof-of-Concept Study","volume":"45","author":"Cristoferi","year":"2025","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"101775","DOI":"10.1016\/j.clinre.2021.101775","article-title":"Non-invasive diagnosis and follow-up of primary sclerosing cholangitis","volume":"46","author":"Potier","year":"2022","journal-title":"Clin. Res. Hepatol. Gastroenterol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"970\u2013979; quiz e15-16","DOI":"10.1053\/j.gastro.2013.12.030","article-title":"Baseline values and changes in liver stiffness measured by transient elastography are associated with severity of fibrosis and outcomes of patients with primary sclerosing cholangitis","volume":"146","author":"Corpechot","year":"2014","journal-title":"Gastroenterology"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1055\/a-2511-3422","article-title":"Surveillance of primary sclerosing cholangitis\u2014A comparison of scheduled or on-demand ERCP with annual MRI surveillance: A multicenter study","volume":"57","author":"Molinaro","year":"2025","journal-title":"Endoscopy"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1055\/s-0034-1391845","article-title":"Prospective evaluation of the clinical utility of single-operator peroral cholangioscopy in patients with primary sclerosing cholangitis","volume":"47","author":"Arnelo","year":"2015","journal-title":"Endoscopy"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1053\/j.gastro.2018.05.034","article-title":"No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients with Primary Sclerosing Cholangitis","volume":"155","author":"Ponsioen","year":"2018","journal-title":"Gastroenterology"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"2372257","DOI":"10.1155\/2022\/2372257","article-title":"ERCP-Related Adverse Events in Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis","volume":"2022","author":"Natt","year":"2022","journal-title":"Can. J. Gastroenterol. Hepatol."},{"key":"ref_82","doi-asserted-by":"crossref","unstructured":"Schmeltzer, P.A., and Russo, M.W. (2021). Systematic Review of Prognostic Models Compared to the Mayo Risk Score for Primary Sclerosing Cholangitis. J. Clin. Med., 10.","DOI":"10.3390\/jcm10194476"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1002\/hep.30085","article-title":"Primary Sclerosing Cholangitis Risk Estimate Tool (PREsTo) Predicts Outcomes of the Disease: A Derivation and Validation Study Using Machine Learning","volume":"71","author":"Eaton","year":"2020","journal-title":"Hepatology"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"2120","DOI":"10.1002\/hep.30479","article-title":"Factors Associated with Outcomes of Patients with Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System","volume":"69","author":"Goode","year":"2019","journal-title":"Hepatology"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1864","DOI":"10.1136\/gutjnl-2016-313681","article-title":"A novel prognostic model for transplant-free survival in primary sclerosing cholangitis","volume":"67","author":"Wang","year":"2018","journal-title":"Gut"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"100328","DOI":"10.1016\/j.jhepr.2021.100328","article-title":"Fluctuating biomarkers in primary sclerosing cholangitis: A longitudinal comparison of alkaline phosphatase, liver stiffness, and ELF","volume":"3","author":"Fossdal","year":"2021","journal-title":"JHEP Rep. Innov. Hepatol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1097\/MOG.0b013e3283292383","article-title":"A conceptual proposal for staging ductal cholangiocarcinoma","volume":"25","author":"Blechacz","year":"2009","journal-title":"Curr. Opin. Gastroenterol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1002\/hep.24103","article-title":"Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis","volume":"53","author":"Lankisch","year":"2011","journal-title":"Hepatology"},{"key":"ref_89","doi-asserted-by":"crossref","unstructured":"Nishida, N. (2025). Biomarkers and Management of Cholangiocarcinoma: Unveiling New Horizons for Precision Therapy. Cancers, 17.","DOI":"10.20944\/preprints202501.1720.v1"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"2338","DOI":"10.1002\/hep.29730","article-title":"Surveillance for hepatobiliary cancers in patients with primary sclerosing cholangitis","volume":"67","author":"Ali","year":"2018","journal-title":"Hepatology"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1080\/17474124.2021.1890584","article-title":"Liver transplantation for perihilar cholangiocarcinoma: Patient selection and outcomes","volume":"15","author":"Acher","year":"2021","journal-title":"Expert. Rev. Gastroenterol. Hepatol."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"2500","DOI":"10.1056\/NEJMc1613273","article-title":"Primary Sclerosing Cholangitis","volume":"375","author":"Valente","year":"2016","journal-title":"N. Engl. J. Med."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1111\/apt.13817","article-title":"Systematic review with meta-analysis: Endoscopic retrograde cholangiopancreatography-based modalities for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis","volume":"44","author":"Njei","year":"2016","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1842","DOI":"10.1002\/hep.24570","article-title":"Cancer surveillance in patients with primary sclerosing cholangitis","volume":"54","author":"Razumilava","year":"2011","journal-title":"Hepatology"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1002\/hep.22441","article-title":"Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis","volume":"48","author":"Charatcharoenwitthaya","year":"2008","journal-title":"Hepatology"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1016\/j.cgh.2011.02.007","article-title":"Many patients with primary sclerosing cholangitis and increased serum levels of carbohydrate antigen 19-9 do not have cholangiocarcinoma","volume":"9","author":"Sinakos","year":"2011","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"5883","DOI":"10.21873\/anticanres.12931","article-title":"Relationship Between CA 19.9 and the Lewis Phenotype: Options to Improve Diagnostic Efficiency","volume":"38","author":"Santos","year":"2018","journal-title":"Anticancer. Res."},{"key":"ref_98","first-page":"54","article-title":"Reference values and biological variation for tumor marker CA 19-9 in serum for different Lewis and secretor genotypes and evaluation of secretor and Lewis genotyping in a Caucasian population","volume":"45","author":"Vestergaard","year":"1999","journal-title":"Clin. Chem."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1053\/j.gastro.2016.06.021","article-title":"Prevalence of Sclerosing Cholangitis Detected by Magnetic Resonance Cholangiography in Patients with Long-term Inflammatory Bowel Disease","volume":"151","author":"Lunder","year":"2016","journal-title":"Gastroenterology"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"1825","DOI":"10.1093\/ecco-jcc\/jjac090","article-title":"Increased Risk of Non-conventional and Invisible Dysplasias in Patients with Primary Sclerosing Cholangitis and Inflammatory Bowel Disease","volume":"16","author":"Zhang","year":"2022","journal-title":"J. Crohns Colitis"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1055\/a-1671-6336","article-title":"Endoscopic tissue sampling\u2014Part 2: Lower gastrointestinal tract. European Society of Gastrointestinal Endoscopy (ESGE) Guideline","volume":"53","author":"Pouw","year":"2021","journal-title":"Endoscopy"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1097\/HEP.0000000000001351","article-title":"Cirrhosis and age are key determinants of HCC risk in individuals with primary sclerosing cholangitis: A multicenter longitudinal cohort study","volume":"83","author":"Holmer","year":"2026","journal-title":"Hepatology"},{"key":"ref_103","first-page":"e70312","article-title":"Ultrasound for the Detection of Gallbladder Malignancy in Primary Sclerosing Cholangitis","volume":"45","author":"Wiegard","year":"2025","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_104","doi-asserted-by":"crossref","unstructured":"Carbone, M., Della Penna, A., Mazzarelli, C., De Martin, E., Villard, C., Bergquist, A., Line, P.D., Neuberger, J.M., Al-Shakhshir, S., and Trivedi, P.J. (2023). Liver Transplantation for Primary Sclerosing Cholangitis (PSC) with or Without Inflammatory Bowel Disease (IBD)-A European Society of Organ Transplantation (ESOT) Consensus Statement. Transpl. Int. Off. J. Eur. Soc. Organ Transplant., 36.","DOI":"10.3389\/ti.2023.11729"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1111\/j.1432-2277.2010.01108.x","article-title":"Liver transplantation for cholangiocarcinoma","volume":"23","author":"Rosen","year":"2010","journal-title":"Transplant. Int. Off. J. Eur. Soc. Organ. Transplant."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1016\/j.jhep.2019.06.012","article-title":"Validation, clinical utility and limitations of the Amsterdam-Oxford model for primary sclerosing cholangitis","volume":"71","author":"Goet","year":"2019","journal-title":"J. Hepatol."},{"key":"ref_107","first-page":"1412","article-title":"Liver transplantation for incidental cholangiocarcinoma: Analysis of the Canadian experience","volume":"11","author":"Ghali","year":"2005","journal-title":"Liver Transplant. Off. Publ. Am. Assoc. Study Liver Dis. Int. Liver Transplant. Soc."},{"key":"ref_108","first-page":"1023","article-title":"Liver transplantation for cholangiocellular carcinoma: Analysis of a single-center experience and review of the literature","volume":"7","author":"Shimoda","year":"2001","journal-title":"Liver Transplant. Off. Publ. Am. Assoc. Study Liver Dis. Int. Liver Transplant. Soc."},{"key":"ref_109","first-page":"696","article-title":"Liver transplantation and whipple surgery combined with chemoradiotherapy for treatment of hilar cholangiocarcinoma in patients with primary sclerosing cholangitis","volume":"21","author":"Nikeghbalian","year":"2015","journal-title":"Liver Transplant. Off. Publ. Am. Assoc. Study Liver Dis. Int. Liver Transplant. Soc."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1111\/apt.15148","article-title":"Systematic review with meta-analysis: Risk factors for recurrent primary sclerosing cholangitis after liver transplantation","volume":"49","author":"Steenstraten","year":"2019","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_111","first-page":"769","article-title":"Outcomes of liver retransplantation in patients with primary sclerosing cholangitis","volume":"23","author":"Henson","year":"2017","journal-title":"Liver Transplant. Off. Publ. Am. Assoc. Study Liver Dis. Int. Liver Transplant. Soc."},{"key":"ref_112","first-page":"e16214","article-title":"Outcome of Liver Retransplantation in Patients with Primary Sclerosing Cholangitis","volume":"45","author":"Molinaro","year":"2025","journal-title":"Liver Int. Off. J. Int. Assoc. Study Liver"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"5447","DOI":"10.1038\/s41598-025-87279-y","article-title":"Artificial intelligence for automatic diagnosis and pleomorphic morphological characterization of malignant biliary strictures using digital cholangioscopy","volume":"15","author":"Mascarenhas","year":"2025","journal-title":"Sci. Rep."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.gie.2021.08.027","article-title":"Artificial intelligence for automatic diagnosis of biliary stricture malignancy status in single-operator cholangioscopy: A pilot study","volume":"95","author":"Saraiva","year":"2022","journal-title":"Gastrointest. Endosc."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1002\/hep.30470","article-title":"Bile-Derived Organoids from Patients with Primary Sclerosing Cholangitis Recapitulate Their Inflammatory Immune Profile","volume":"70","author":"Soroka","year":"2019","journal-title":"Hepatology"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1126\/science.aaz6964","article-title":"Cholangiocyte organoids can repair bile ducts after transplantation in the human liver","volume":"371","author":"Sampaziotis","year":"2021","journal-title":"Science"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/3\/1149\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,4]],"date-time":"2026-02-04T05:24:56Z","timestamp":1770182696000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/3\/1149"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,2]]},"references-count":116,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,2]]}},"alternative-id":["jcm15031149"],"URL":"https:\/\/doi.org\/10.3390\/jcm15031149","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,2]]}}}