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Primary sclerosing cholangitis(PSC)

MedGen UID:
107565
•Concept ID:
C0566602
•
Disease or Syndrome
Synonym: PSC
SNOMED CT: Primary sclerosing cholangitis (197441003); PSC - Primary sclerosing cholangitis (197441003)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0013433
OMIM®: 613806
Orphanet: ORPHA171

Definition

Primary sclerosing cholangitis (PSC) is a slowly progressive cholestatic liver disease characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension. It is a major indication for liver transplantation (Sheth et al., 2003). Approximately 75 to 80% of PSC cases are associated with inflammatory bowel disease (IBD; see 266600), and 2.5 to 7.5% of patients with IBD develop PSC (Lee and Kaplan, 1995). [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary sclerosing cholangitis
Follow this link to review classifications for Primary sclerosing cholangitis in Orphanet.

Professional guidelines

PubMed

Extraintestinal Manifestations of Inflammatory Bowel Disease: Current Concepts, Treatment, and Implications for Disease Management.
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article
ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries.
Kwo PY, Cohen SM, Lim JK
Am J Gastroenterol 2017 Jan;112(1):18-35. Epub 2016 Dec 20 doi: 10.1038/ajg.2016.517. PMID: 27995906
Pathogenesis, diagnosis, and management of cholangiocarcinoma.
Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article

Recent clinical studies

Etiology

Recent Advances in the Management of Primary Sclerosing Cholangitis.
Assis DN, Bowlus CL
Clin Gastroenterol Hepatol 2023 Jul;21(8):2065-2075. Epub 2023 Apr 19 doi: 10.1016/j.cgh.2023.04.004. PMID: 37084929
Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.
Barberio B, Massimi D, Cazzagon N, Zingone F, Ford AC, Savarino EV
Gastroenterology 2021 Dec;161(6):1865-1877. Epub 2021 Aug 20 doi: 10.1053/j.gastro.2021.08.032. PMID: 34425093
Primary sclerosing cholangitis.
Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M
Lancet 2018 Jun 23;391(10139):2547-2559. Epub 2018 Feb 13 doi: 10.1016/S0140-6736(18)30300-3. PMID: 29452711
Primary sclerosing cholangitis - a comprehensive review.
Karlsen TH, Folseraas T, Thorburn D, Vesterhus M
J Hepatol 2017 Dec;67(6):1298-1323. Epub 2017 Aug 10 doi: 10.1016/j.jhep.2017.07.022. PMID: 28802875
Primary sclerosing cholangitis.
Hirschfield GM, Karlsen TH, Lindor KD, Adams DH
Lancet 2013 Nov 9;382(9904):1587-99. Epub 2013 Jun 28 doi: 10.1016/S0140-6736(13)60096-3. PMID: 23810223

Diagnosis

Inflammatory bowel disease and primary sclerosing cholangitis: One disease or two?
van Munster KN, Bergquist A, Ponsioen CY
J Hepatol 2024 Jan;80(1):155-168. Epub 2023 Nov 6 doi: 10.1016/j.jhep.2023.09.031. PMID: 37940453
Recent Advances in the Management of Primary Sclerosing Cholangitis.
Assis DN, Bowlus CL
Clin Gastroenterol Hepatol 2023 Jul;21(8):2065-2075. Epub 2023 Apr 19 doi: 10.1016/j.cgh.2023.04.004. PMID: 37084929
Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.
Barberio B, Massimi D, Cazzagon N, Zingone F, Ford AC, Savarino EV
Gastroenterology 2021 Dec;161(6):1865-1877. Epub 2021 Aug 20 doi: 10.1053/j.gastro.2021.08.032. PMID: 34425093
Primary sclerosing cholangitis.
Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M
Lancet 2018 Jun 23;391(10139):2547-2559. Epub 2018 Feb 13 doi: 10.1016/S0140-6736(18)30300-3. PMID: 29452711
Primary sclerosing cholangitis - a comprehensive review.
Karlsen TH, Folseraas T, Thorburn D, Vesterhus M
J Hepatol 2017 Dec;67(6):1298-1323. Epub 2017 Aug 10 doi: 10.1016/j.jhep.2017.07.022. PMID: 28802875

Therapy

IgG4-related cholangitis - a mimicker of fibrosing and malignant cholangiopathies.
Kersten R, Trampert DC, Herta T, Hubers LM, Maillette de Buy Wenniger LJ, Verheij J, van de Graaf SFJ, Beuers U
J Hepatol 2023 Dec;79(6):1502-1523. Epub 2023 Aug 18 doi: 10.1016/j.jhep.2023.08.005. PMID: 37598939
Extraintestinal Manifestations of Inflammatory Bowel Disease: Current Concepts, Treatment, and Implications for Disease Management.
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article
Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature.
Kawaguchi T, Arinaga-Hino T, Morishige S, Mizuochi S, Abe M, Kunitake K, Sano T, Amano K, Kuwahara R, Ide T, Nagafuji K, Torimura T
Clin J Gastroenterol 2021 Feb;14(1):330-335. Epub 2020 Oct 10 doi: 10.1007/s12328-020-01256-8. PMID: 33038000
Cholangiocyte-Derived Exosomal Long Noncoding RNA H19 Promotes Hepatic Stellate Cell Activation and Cholestatic Liver Fibrosis.
Liu R, Li X, Zhu W, Wang Y, Zhao D, Wang X, Gurley EC, Liang G, Chen W, Lai G, Pandak WM, Robert Lippman H, Bajaj JS, Hylemon PB, Zhou H
Hepatology 2019 Oct;70(4):1317-1335. Epub 2019 May 24 doi: 10.1002/hep.30662. PMID: 30985008Free PMC Article
Autoimmune hepatitis.
Sahebjam F, Vierling JM
Front Med 2015 Jun;9(2):187-219. Epub 2015 Mar 6 doi: 10.1007/s11684-015-0386-y. PMID: 25749982

Prognosis

Management and follow-up of gallbladder polyps: updated joint guidelines between the ESGAR, EAES, EFISDS and ESGE.
Foley KG, Lahaye MJ, Thoeni RF, Soltes M, Dewhurst C, Barbu ST, Vashist YK, Rafaelsen SR, Arvanitakis M, Perinel J, Wiles R, Roberts SA
Eur Radiol 2022 May;32(5):3358-3368. Epub 2021 Dec 17 doi: 10.1007/s00330-021-08384-w. PMID: 34918177Free PMC Article
Recent advances in clinical practice: epidemiology of autoimmune liver diseases.
Trivedi PJ, Hirschfield GM
Gut 2021 Oct;70(10):1989-2003. Epub 2021 Jul 15 doi: 10.1136/gutjnl-2020-322362. PMID: 34266966
Cholangiocellular Carcinoma.
Vogel A, Saborowski A
Digestion 2017;95(3):181-185. Epub 2017 Mar 14 doi: 10.1159/000454763. PMID: 28288474
Pathogenesis, diagnosis, and management of cholangiocarcinoma.
Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article
Transplantation for cholangiocarcinoma.
Masuoka HC, Rosen CB
Clin Liver Dis 2011 Nov;15(4):699-715. doi: 10.1016/j.cld.2011.08.004. PMID: 22032524

Clinical prediction guides

Altered faecal microbiome and metabolome in IgG4-related sclerosing cholangitis and primary sclerosing cholangitis.
Liu Q, Li B, Li Y, Wei Y, Huang B, Liang J, You Z, Li Y, Qian Q, Wang R, Zhang J, Chen R, Lyu Z, Chen Y, Shi M, Xiao X, Wang Q, Miao Q, Fang JY, Gershwin ME, Lian M, Ma X, Tang R
Gut 2022 May;71(5):899-909. Epub 2021 May 25 doi: 10.1136/gutjnl-2020-323565. PMID: 34035120
Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.
Barberio B, Massimi D, Cazzagon N, Zingone F, Ford AC, Savarino EV
Gastroenterology 2021 Dec;161(6):1865-1877. Epub 2021 Aug 20 doi: 10.1053/j.gastro.2021.08.032. PMID: 34425093
Extraintestinal Manifestations of Inflammatory Bowel Disease: Current Concepts, Treatment, and Implications for Disease Management.
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article
Prednisolone-responsive primary sclerosing cholangitis with autoimmune hemolytic anemia: a case report and review of the literature.
Kawaguchi T, Arinaga-Hino T, Morishige S, Mizuochi S, Abe M, Kunitake K, Sano T, Amano K, Kuwahara R, Ide T, Nagafuji K, Torimura T
Clin J Gastroenterol 2021 Feb;14(1):330-335. Epub 2020 Oct 10 doi: 10.1007/s12328-020-01256-8. PMID: 33038000
Ulcerative colitis-associated colorectal cancer.
Yashiro M
World J Gastroenterol 2014 Nov 28;20(44):16389-97. doi: 10.3748/wjg.v20.i44.16389. PMID: 25469007Free PMC Article

Recent systematic reviews

Cholestatic Pruritus Treatments in Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: A Systematic Literature Review.
Smith HT, de Souza AR, Thompson AH, McLaughlin MM, Dever JJ, Myers JA, Chen JV
Dig Dis Sci 2023 Jun;68(6):2710-2730. Epub 2023 Mar 18 doi: 10.1007/s10620-023-07862-z. PMID: 36933112Free PMC Article
Systematic review: microbial manipulation as therapy for primary sclerosing cholangitis.
Bogatic D, Bryant RV, Lynch KD, Costello SP
Aliment Pharmacol Ther 2023 Jan;57(1):23-36. Epub 2022 Nov 2 doi: 10.1111/apt.17251. PMID: 36324251Free PMC Article
Epidemiology, Natural History, and Outcomes of Primary Sclerosing Cholangitis: A Systematic Review of Population-based Studies.
Trivedi PJ, Bowlus CL, Yimam KK, Razavi H, Estes C
Clin Gastroenterol Hepatol 2022 Aug;20(8):1687-1700.e4. Epub 2021 Aug 30 doi: 10.1016/j.cgh.2021.08.039. PMID: 34474162
Prevalence of Primary Sclerosing Cholangitis in Patients With Inflammatory Bowel Disease: A Systematic Review and Meta-analysis.
Barberio B, Massimi D, Cazzagon N, Zingone F, Ford AC, Savarino EV
Gastroenterology 2021 Dec;161(6):1865-1877. Epub 2021 Aug 20 doi: 10.1053/j.gastro.2021.08.032. PMID: 34425093
Prognostic Factors for Advanced Colorectal Neoplasia in Inflammatory Bowel Disease: Systematic Review and Meta-analysis.
Wijnands AM, de Jong ME, Lutgens MWMD, Hoentjen F, Elias SG, Oldenburg B; Dutch Initiative on Crohn and Colitis (ICC)
Gastroenterology 2021 Apr;160(5):1584-1598. Epub 2020 Dec 29 doi: 10.1053/j.gastro.2020.12.036. PMID: 33385426

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