U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

IgG4-related sclerosing cholangitis

MedGen UID:
927778
Concept ID:
C4302109
Disease or Syndrome
Synonym: Immunoglobulin G4-related sclerosing cholangitis
SNOMED CT: IgG4-related sclerosing cholangitis (722870008); Immunoglobulin G4-related sclerosing cholangitis (722870008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018645
Orphanet: ORPHA447764

Definition

A rare systemic autoimmune disease characterized by cholestasis and diffuse cholangiographic abnormalities with circular and symmetrical bile duct wall thickening, and elevated serum IgG4 levels. Characteristic histopathological findings include dense infiltration of IgG4-positive plasma cells and extensive fibrosis in the bile duct wall. A marked response to steroid therapy is typical. Patients present with jaundice, cholangitis, pruritis, and sometimes associated findings of autoimmune pancreatitis, sialadenitis, and retroperitoneal fibrosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgG4-related sclerosing cholangitis

Professional guidelines

PubMed

Naitoh I, Nakazawa T
Gut Liver 2022 Jan 15;16(1):28-36. doi: 10.5009/gnl210116. PMID: 34380781Free PMC Article
Jha S, Sureka B, Khera PS
AJR Am J Roentgenol 2020 Aug;215(2):W23. doi: 10.2214/AJR.20.22765. PMID: 32749881
Kamisawa T, Nakazawa T, Tazuma S, Zen Y, Tanaka A, Ohara H, Muraki T, Inui K, Inoue D, Nishino T, Naitoh I, Itoi T, Notohara K, Kanno A, Kubota K, Hirano K, Isayama H, Shimizu K, Tsuyuguchi T, Shimosegawa T, Kawa S, Chiba T, Okazaki K, Takikawa H, Kimura W, Unno M, Yoshida M
J Hepatobiliary Pancreat Sci 2019 Jan;26(1):9-42. Epub 2019 Jan 18 doi: 10.1002/jhbp.596. PMID: 30575336Free PMC Article

Recent clinical studies

Etiology

Kurita Y, Fujita Y, Sekino Y, Watanabe S, Iwasaki A, Kagawa K, Tanida E, Yagi S, Hasegawa S, Sato T, Hosono K, Kato S, Kobayashi N, Ichikawa Y, Endo I, Nakajima A, Kubota K
J Hepatobiliary Pancreat Sci 2021 Jun;28(6):524-532. Epub 2021 May 24 doi: 10.1002/jhbp.957. PMID: 33931982
Kamisawa T, Zen Y, Nakazawa T, Okazaki K
Lancet Gastroenterol Hepatol 2018 Aug;3(8):575-585. doi: 10.1016/S2468-1253(18)30121-3. PMID: 30047448
Goodchild G, Pereira SP, Webster G
Korean J Intern Med 2018 Sep;33(5):841-850. Epub 2018 Jul 27 doi: 10.3904/kjim.2018.018. PMID: 30045615Free PMC Article
Culver EL, Chapman RW
Nat Rev Gastroenterol Hepatol 2016 Oct;13(10):601-12. Epub 2016 Sep 14 doi: 10.1038/nrgastro.2016.132. PMID: 27625195
Zen Y, Kawakami H, Kim JH
J Gastroenterol 2016 Apr;51(4):295-312. Epub 2016 Jan 27 doi: 10.1007/s00535-016-1163-7. PMID: 26817943

Diagnosis

Goyal S, Sakhuja P
Indian J Pathol Microbiol 2021 Jun;64(Supplement):S149-S159. doi: 10.4103/ijpm.ijpm_59_21. PMID: 34135159
Nayak HK, Bhat SJ, Panigrahi MK, Chouhan I, Kumar C, Samal SC
Indian J Gastroenterol 2020 Dec;39(6):614-618. Epub 2020 Nov 6 doi: 10.1007/s12664-020-01102-2. PMID: 33156480
Kamisawa T, Nakazawa T, Tazuma S, Zen Y, Tanaka A, Ohara H, Muraki T, Inui K, Inoue D, Nishino T, Naitoh I, Itoi T, Notohara K, Kanno A, Kubota K, Hirano K, Isayama H, Shimizu K, Tsuyuguchi T, Shimosegawa T, Kawa S, Chiba T, Okazaki K, Takikawa H, Kimura W, Unno M, Yoshida M
J Hepatobiliary Pancreat Sci 2019 Jan;26(1):9-42. Epub 2019 Jan 18 doi: 10.1002/jhbp.596. PMID: 30575336Free PMC Article
Goodchild G, Pereira SP, Webster G
Korean J Intern Med 2018 Sep;33(5):841-850. Epub 2018 Jul 27 doi: 10.3904/kjim.2018.018. PMID: 30045615Free PMC Article
Nakazawa T, Shimizu S, Naitoh I
Semin Liver Dis 2016 Aug;36(3):216-28. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584321. PMID: 27466792

Therapy

Moon SH, Kim MH
Korean J Gastroenterol 2022 Sep 25;80(3):107-114. doi: 10.4166/kjg.2022.102. PMID: 36156034
Kamisawa T, Zen Y, Nakazawa T, Okazaki K
Lancet Gastroenterol Hepatol 2018 Aug;3(8):575-585. doi: 10.1016/S2468-1253(18)30121-3. PMID: 30047448
Nakazawa T, Shimizu S, Naitoh I
Semin Liver Dis 2016 Aug;36(3):216-28. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584321. PMID: 27466792
Zen Y, Kawakami H, Kim JH
J Gastroenterol 2016 Apr;51(4):295-312. Epub 2016 Jan 27 doi: 10.1007/s00535-016-1163-7. PMID: 26817943
Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T
World J Gastroenterol 2013 Nov 21;19(43):7661-70. doi: 10.3748/wjg.v19.i43.7661. PMID: 24282356Free PMC Article

Prognosis

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
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
Goyal S, Sakhuja P
Indian J Pathol Microbiol 2021 Jun;64(Supplement):S149-S159. doi: 10.4103/ijpm.ijpm_59_21. PMID: 34135159
Nakazawa T, Shimizu S, Naitoh I
Semin Liver Dis 2016 Aug;36(3):216-28. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584321. PMID: 27466792
Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T
World J Gastroenterol 2013 Nov 21;19(43):7661-70. doi: 10.3748/wjg.v19.i43.7661. PMID: 24282356Free PMC Article

Clinical prediction guides

Capurso G, Pedica F, Palumbo D, Della-Torre E
Minerva Gastroenterol (Torino) 2023 Mar;69(1):23-49. Epub 2020 Dec 3 doi: 10.23736/S2724-5895.20.02794-4. PMID: 33267565
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
Notohara K
J Med Ultrason (2001) 2021 Oct;48(4):581-594. Epub 2021 Oct 20 doi: 10.1007/s10396-021-01148-5. PMID: 34669070
Nakazawa T, Shimizu S, Naitoh I
Semin Liver Dis 2016 Aug;36(3):216-28. Epub 2016 Jul 28 doi: 10.1055/s-0036-1584321. PMID: 27466792
Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T
World J Gastroenterol 2013 Nov 21;19(43):7661-70. doi: 10.3748/wjg.v19.i43.7661. PMID: 24282356Free PMC Article

Recent systematic reviews

Lanzillotta M, Della-Torre E, Wallace ZS, Stone JH, Karadag O, Fernández-Codina A, Arcidiacono PG, Falconi M, Dagna L, Capurso G
Pancreatology 2021 Oct;21(7):1395-1401. Epub 2021 Jul 3 doi: 10.1016/j.pan.2021.06.009. PMID: 34244040

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...