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Granulomatous cholangitis

MedGen UID:
1386271
•Concept ID:
C4476897
•
Disease or Syndrome
HPO: HP:0030988

Definition

Cholangitis characterized by the accumulation of granulomas. Granulomas are aggregates of modified macrophages (epithelioid cells) and other inflammatory cells that accumulate after chronic exposure to antigens. The underlying trigger may be exposure to noxious agents that cannot be biochemically degraded or to immune dysfunction. The ultimate result is a release of a variety cytokines that stimulate mononuclear cells that fuse to form multinucleated giant cells with a surrounding rim of lymphocytes and fibroblasts. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGranulomatous cholangitis

Professional guidelines

PubMed

Histopathologic findings in lung biopsies from patients with primary biliary cholangitis.
Lee HE, Churg A, Ryu JH, Bilawich AM, Larsen BT, Tazelaar HD, Yi ES
Hum Pathol 2018 Dec;82:177-186. Epub 2018 Jul 29 doi: 10.1016/j.humpath.2018.07.021. PMID: 30067952
An Update on the Treatment and Follow-up of Patients with Primary Biliary Cholangitis.
Burman BE, Jhaveri MA, Kowdley KV
Clin Liver Dis 2017 Nov;21(4):709-723. Epub 2017 Jul 26 doi: 10.1016/j.cld.2017.06.005. PMID: 28987258
The diagnosis and treatment of pouchitis in inflammatory bowel disease.
Cheifetz A, Itzkowitz S
J Clin Gastroenterol 2004 May-Jun;38(5 Suppl 1):S44-50. doi: 10.1097/01.mcg.0000124001.93146.ef. PMID: 15115932

Recent clinical studies

Etiology

Benign hilar bile duct strictures resected as perihilar cholangiocarcinoma.
Otsuka S, Ebata T, Yokoyama Y, Igami T, Mizuno T, Yamaguchi J, Onoe S, Watanabe N, Shimoyama Y, Nagino M
Br J Surg 2019 Oct;106(11):1504-1511. Epub 2019 Aug 6 doi: 10.1002/bjs.11257. PMID: 31386198
Biliary features in liver histology of children with autoimmune liver disease.
Di Giorgio A, D'Adda A, Marseglia A, Sonzogni A, Licini L, Nicastro E, D'Antiga L
Hepatol Int 2019 Jul;13(4):510-518. Epub 2019 May 8 doi: 10.1007/s12072-019-09948-1. PMID: 31069759
Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants.
Hirschfield GM, Liu X, Xu C, Lu Y, Xie G, Lu Y, Gu X, Walker EJ, Jing K, Juran BD, Mason AL, Myers RP, Peltekian KM, Ghent CN, Coltescu C, Atkinson EJ, Heathcote EJ, Lazaridis KN, Amos CI, Siminovitch KA
N Engl J Med 2009 Jun 11;360(24):2544-55. Epub 2009 May 20 doi: 10.1056/NEJMoa0810440. PMID: 19458352Free PMC Article
Granulomas in primary sclerosing cholangitis.
Ludwig J, Colina F, Poterucha JJ
Liver 1995 Dec;15(6):307-12. doi: 10.1111/j.1600-0676.1995.tb00690.x. PMID: 8609810
Enzyme patterns in human endocytotic multinucleate giant cells--a histochemical study.
Elleder M
Acta Histochem 1986;79(1):1-10. doi: 10.1016/S0065-1281(86)80091-5. PMID: 2874682

Diagnosis

Granulomatous cholangitis mimicking hilar cholangiocarcinoma: a case report.
Fujisaki S, Takashina M, Sakurai KI, Tomita R, Takayama T
BMC Gastroenterol 2020 Nov 4;20(1):369. doi: 10.1186/s12876-020-01519-1. PMID: 33148196Free PMC Article
Benign hilar bile duct strictures resected as perihilar cholangiocarcinoma.
Otsuka S, Ebata T, Yokoyama Y, Igami T, Mizuno T, Yamaguchi J, Onoe S, Watanabe N, Shimoyama Y, Nagino M
Br J Surg 2019 Oct;106(11):1504-1511. Epub 2019 Aug 6 doi: 10.1002/bjs.11257. PMID: 31386198
Biliary features in liver histology of children with autoimmune liver disease.
Di Giorgio A, D'Adda A, Marseglia A, Sonzogni A, Licini L, Nicastro E, D'Antiga L
Hepatol Int 2019 Jul;13(4):510-518. Epub 2019 May 8 doi: 10.1007/s12072-019-09948-1. PMID: 31069759
Sarcoidosis of the liver and bile ducts.
Ishak KG
Mayo Clin Proc 1998 May;73(5):467-72. doi: 10.1016/S0025-6196(11)63733-6. PMID: 9581591
Granulomas in primary sclerosing cholangitis.
Ludwig J, Colina F, Poterucha JJ
Liver 1995 Dec;15(6):307-12. doi: 10.1111/j.1600-0676.1995.tb00690.x. PMID: 8609810

Therapy

Biliary features in liver histology of children with autoimmune liver disease.
Di Giorgio A, D'Adda A, Marseglia A, Sonzogni A, Licini L, Nicastro E, D'Antiga L
Hepatol Int 2019 Jul;13(4):510-518. Epub 2019 May 8 doi: 10.1007/s12072-019-09948-1. PMID: 31069759
Intrahepatic cholangitis and arteritis after transcatheter arterial embolization in a patient with tumor-like lesion-associated autoimmune hepatitis.
Iwata M, Sasaki M, Harada K, Kaneko S, Kobayashi K, Adachi K, Sasaki M, Nakanuma Y
Pathol Res Pract 2001;197(1):59-63. doi: 10.1078/0344-0338-00011. PMID: 11209818
Primary autoimmune cholangitis. An alternative to antimitochondrial antibody-negative primary biliary cirrhosis.
Taylor SL, Dean PJ, Riely CA
Am J Surg Pathol 1994 Jan;18(1):91-9. PMID: 8279631

Prognosis

Granulomatous cholangitis mimicking hilar cholangiocarcinoma: a case report.
Fujisaki S, Takashina M, Sakurai KI, Tomita R, Takayama T
BMC Gastroenterol 2020 Nov 4;20(1):369. doi: 10.1186/s12876-020-01519-1. PMID: 33148196Free PMC Article
Biliary features in liver histology of children with autoimmune liver disease.
Di Giorgio A, D'Adda A, Marseglia A, Sonzogni A, Licini L, Nicastro E, D'Antiga L
Hepatol Int 2019 Jul;13(4):510-518. Epub 2019 May 8 doi: 10.1007/s12072-019-09948-1. PMID: 31069759
Primary autoimmune cholangitis. An alternative to antimitochondrial antibody-negative primary biliary cirrhosis.
Taylor SL, Dean PJ, Riely CA
Am J Surg Pathol 1994 Jan;18(1):91-9. PMID: 8279631
Primary lymphoma of the liver: clinical and pathological features of 10 patients.
Anthony PP, Sarsfield P, Clarke T
J Clin Pathol 1990 Dec;43(12):1007-13. doi: 10.1136/jcp.43.12.1007. PMID: 2266172Free PMC Article
Enzyme patterns in human endocytotic multinucleate giant cells--a histochemical study.
Elleder M
Acta Histochem 1986;79(1):1-10. doi: 10.1016/S0065-1281(86)80091-5. PMID: 2874682

Clinical prediction guides

Biliary features in liver histology of children with autoimmune liver disease.
Di Giorgio A, D'Adda A, Marseglia A, Sonzogni A, Licini L, Nicastro E, D'Antiga L
Hepatol Int 2019 Jul;13(4):510-518. Epub 2019 May 8 doi: 10.1007/s12072-019-09948-1. PMID: 31069759

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