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Secondary sclerosing cholangitis

MedGen UID:
586530
Concept ID:
C0400978
Disease or Syndrome
Synonym: secondary sclerosing cholangitis
SNOMED CT: Secondary sclerosing cholangitis (197442005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018647
Orphanet: ORPHA447774

Definition

A rare, biliary tract disease characterized by development of sclerosing cholangitis due to a known primary insult to the biliary tree, including infections, autoimmune disease, exposure to toxic agents, obstructive and ischemic injuries. Patients may be initially asymptomatic with only elevated alkaline phosphatase and gamma glutamyltransferase levels. Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSecondary sclerosing cholangitis

Professional guidelines

PubMed

Cazzagon N, Sarcognato S, Catanzaro E, Bonaiuto E, Peviani M, Pezzato F, Motta R
Tomography 2024 Jan 7;10(1):47-65. doi: 10.3390/tomography10010005. PMID: 38250951Free PMC Article
European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver
J Hepatol 2022 Sep;77(3):761-806. Epub 2022 Jun 21 doi: 10.1016/j.jhep.2022.05.011. PMID: 35738507
Imam MH, Talwalkar JA, Lindor KD
Clin Liver Dis 2013 May;17(2):269-77. Epub 2012 Dec 20 doi: 10.1016/j.cld.2012.11.004. PMID: 23540502

Recent clinical studies

Etiology

Gupta T, Sharma H
World J Gastroenterol 2023 Jan 28;29(4):656-669. doi: 10.3748/wjg.v29.i4.656. PMID: 36742164Free PMC Article
Pria HD, Torres US, Faria SC, Velloni FG, Caiado AHM, Tiferes DA, D'Ippolito G
Semin Ultrasound CT MR 2022 Dec;43(6):490-509. Epub 2022 Jun 10 doi: 10.1053/j.sult.2022.06.007. PMID: 36462808
Zhang CC, Sauer P, Rupp C
J Gastroenterol Hepatol 2022 Oct;37(10):2011-2018. Epub 2022 Aug 18 doi: 10.1111/jgh.15977. PMID: 35933581
de 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, Mostafavi N, Helder J, Ponsioen C, Oude Elferink R, van Buuren H, Beuers U; Netherlands Association for the Study of the Liver-Cholestasis Working Group
Gastroenterology 2021 Feb;160(3):734-743.e6. Epub 2020 Oct 5 doi: 10.1053/j.gastro.2020.10.001. PMID: 33031833
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

Cazzagon N, Sarcognato S, Catanzaro E, Bonaiuto E, Peviani M, Pezzato F, Motta R
Tomography 2024 Jan 7;10(1):47-65. doi: 10.3390/tomography10010005. PMID: 38250951Free PMC Article
European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver
J Hepatol 2022 Sep;77(3):761-806. Epub 2022 Jun 21 doi: 10.1016/j.jhep.2022.05.011. PMID: 35738507
Kim TS, Choi DH
Korean J Gastroenterol 2020 Apr 25;75(4):182-187. doi: 10.4166/kjg.2020.75.4.182. PMID: 32326684
Jüngst C, Lammert F
Dig Dis 2013;31(1):152-4. Epub 2013 Jun 17 doi: 10.1159/000347210. PMID: 23797137
Ruemmele P, Hofstaedter F, Gelbmann CM
Nat Rev Gastroenterol Hepatol 2009 May;6(5):287-95. doi: 10.1038/nrgastro.2009.46. PMID: 19404269

Therapy

Bauer U, Pavlova D, Abbassi R, Lahmer T, Geisler F, Schmid RM, Ehmer U
Clin J Gastroenterol 2022 Dec;15(6):1124-1129. Epub 2022 Aug 11 doi: 10.1007/s12328-022-01687-5. PMID: 35953614Free PMC Article
Takinami M, Ono A, Kawabata T, Mamesaya N, Kobayashi H, Omori S, Wakuda K, Kenmotsu H, Naito T, Murakami H, Endo M, Kiyohara Y, Yasui H, Niwakawa M, Takahashi T
Invest New Drugs 2021 Dec;39(6):1716-1723. Epub 2021 May 28 doi: 10.1007/s10637-021-01136-z. PMID: 34046801Free PMC Article
de 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, Mostafavi N, Helder J, Ponsioen C, Oude Elferink R, van Buuren H, Beuers U; Netherlands Association for the Study of the Liver-Cholestasis Working Group
Gastroenterology 2021 Feb;160(3):734-743.e6. Epub 2020 Oct 5 doi: 10.1053/j.gastro.2020.10.001. PMID: 33031833
Strnad P, Tacke F, Koch A, Trautwein C
Nat Rev Gastroenterol Hepatol 2017 Jan;14(1):55-66. Epub 2016 Dec 7 doi: 10.1038/nrgastro.2016.168. PMID: 27924081
Joshi D, Webster GJ
Aliment Pharmacol Ther 2014 Dec;40(11-12):1251-61. Epub 2014 Oct 13 doi: 10.1111/apt.12988. PMID: 25312536

Prognosis

European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu; European Association for the Study of the Liver
J Hepatol 2022 Sep;77(3):761-806. Epub 2022 Jun 21 doi: 10.1016/j.jhep.2022.05.011. PMID: 35738507
Drazilova S, Veseliny E, Lenartova PD, Drazilova D, Gazda J, Grgurevic I, Janicko M, Jarcuska P
Can J Gastroenterol Hepatol 2021;2021:1959832. Epub 2021 Dec 21 doi: 10.1155/2021/1959832. PMID: 34970512Free PMC Article
Brooling J, Leal R
Curr Gastroenterol Rep 2017 Sep;19(9):44. doi: 10.1007/s11894-017-0583-8. PMID: 28752474
Strnad P, Tacke F, Koch A, Trautwein C
Nat Rev Gastroenterol Hepatol 2017 Jan;14(1):55-66. Epub 2016 Dec 7 doi: 10.1038/nrgastro.2016.168. PMID: 27924081
Ruemmele P, Hofstaedter F, Gelbmann CM
Nat Rev Gastroenterol Hepatol 2009 May;6(5):287-95. doi: 10.1038/nrgastro.2009.46. PMID: 19404269

Clinical prediction guides

Cazzagon N, Sarcognato S, Catanzaro E, Bonaiuto E, Peviani M, Pezzato F, Motta R
Tomography 2024 Jan 7;10(1):47-65. doi: 10.3390/tomography10010005. PMID: 38250951Free PMC Article
Horwich BH, Dieterich DT
Clin Liver Dis 2024 Feb;28(1):143-155. Epub 2023 Aug 17 doi: 10.1016/j.cld.2023.07.006. PMID: 37945155
Kim TS, Choi DH
Korean J Gastroenterol 2020 Apr 25;75(4):182-187. doi: 10.4166/kjg.2020.75.4.182. PMID: 32326684
Strnad P, Tacke F, Koch A, Trautwein C
Nat Rev Gastroenterol Hepatol 2017 Jan;14(1):55-66. Epub 2016 Dec 7 doi: 10.1038/nrgastro.2016.168. PMID: 27924081
Velayudham LS, Farrell GC
Expert Opin Drug Saf 2003 May;2(3):287-304. doi: 10.1517/14740338.2.3.287. PMID: 12904107

Recent systematic reviews

Ziogas IA, Kakos CD, Wu WK, Montenovo MI, Matsuoka LK, Zarnegar-Lumley S, Alexopoulos SP
Liver Transpl 2021 Aug;27(8):1181-1190. Epub 2021 Jul 14 doi: 10.1002/lt.25995. PMID: 33484600

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