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Lymphoid cholangitis

MedGen UID:
1376805
•Concept ID:
C4476898
•
Disease or Syndrome
HPO: HP:0030989

Definition

Cholangitis characterized by a close association between duct branches, usually interlobular bile ducts, and lymphocytic aggregates, which may show a follicular arrangement. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphoid 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

Recent clinical studies

Etiology

Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate.
Hu YF, Li SX, Liu HL, Du ZX, Wang SS, Chen MY, Wang L, Xiong QF, Zhong YD, Liu DX, Yang YF
Gut Liver 2024 Sep 15;18(5):867-876. Epub 2024 Apr 16 doi: 10.5009/gnl230468. PMID: 38623061Free PMC Article
Imbalanced distribution of group 2 innate lymphoid cells (ILCs) and ILC precursors in peripheral blood of patients with primary biliary cholangitis.
Li H, Zhan H, Cheng L, Yan S, Wang L, Li Y
Scand J Immunol 2022 Jul;96(1):e13166. Epub 2022 Apr 3 doi: 10.1111/sji.13166. PMID: 35315090
Th1 and Innate Lymphoid Cells Accumulate in Primary Sclerosing Cholangitis-associated Inflammatory Bowel Disease.
Gwela A, Siddhanathi P, Chapman RW, Travis S, Powrie F, Arancibia-Cárcamo CV, Geremia A
J Crohns Colitis 2017 Sep 1;11(9):1124-1134. doi: 10.1093/ecco-jcc/jjx050. PMID: 28383652Free PMC Article
Follicular cholangitis and pancreatitis - clinicopathological features and differential diagnosis of an under-recognized entity.
Zen Y, Ishikawa A, Ogiso S, Heaton N, Portmann B
Histopathology 2012 Jan;60(2):261-9. doi: 10.1111/j.1365-2559.2011.04078.x. PMID: 22211284
Radiology of AIDS in the pediatric patient.
Grattan-Smith D, Harrison LF, Singleton EB
Curr Probl Diagn Radiol 1992 May-Jun;21(3):79-109. doi: 10.1016/0363-0188(92)90038-h. PMID: 1576831

Diagnosis

Th1 and Innate Lymphoid Cells Accumulate in Primary Sclerosing Cholangitis-associated Inflammatory Bowel Disease.
Gwela A, Siddhanathi P, Chapman RW, Travis S, Powrie F, Arancibia-Cárcamo CV, Geremia A
J Crohns Colitis 2017 Sep 1;11(9):1124-1134. doi: 10.1093/ecco-jcc/jjx050. PMID: 28383652Free PMC Article
Increases in IgE, Eosinophils, and Mast Cells Can be Used in Diagnosis and to Predict Relapse of IgG4-Related Disease.
Culver EL, Sadler R, Bateman AC, Makuch M, Cargill T, Ferry B, Aalberse R, Barnes E, Rispens T
Clin Gastroenterol Hepatol 2017 Sep;15(9):1444-1452.e6. Epub 2017 Feb 20 doi: 10.1016/j.cgh.2017.02.007. PMID: 28223204Free PMC Article
Unmet challenges in immune-mediated hepatobiliary diseases.
Beuers U, Gershwin ME
Clin Rev Allergy Immunol 2015 Jun;48(2-3):127-31. doi: 10.1007/s12016-015-8484-9. PMID: 25820618
Liver abnormalities in connective tissue diseases.
De Santis M, Crotti C, Selmi C
Best Pract Res Clin Gastroenterol 2013 Aug;27(4):543-51. doi: 10.1016/j.bpg.2013.06.016. PMID: 24090941
Radiology of AIDS in the pediatric patient.
Grattan-Smith D, Harrison LF, Singleton EB
Curr Probl Diagn Radiol 1992 May-Jun;21(3):79-109. doi: 10.1016/0363-0188(92)90038-h. PMID: 1576831

Therapy

Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate.
Hu YF, Li SX, Liu HL, Du ZX, Wang SS, Chen MY, Wang L, Xiong QF, Zhong YD, Liu DX, Yang YF
Gut Liver 2024 Sep 15;18(5):867-876. Epub 2024 Apr 16 doi: 10.5009/gnl230468. PMID: 38623061Free PMC Article
The Incidence of Pouch Neoplasia Following Ileal Pouch-Anal Anastomosis in Patients With Inflammatory Bowel Disease.
Urquhart SA, Comstock BP, Jin MF, Day CN, Eaton JE, Harmsen WS, Raffals LE, Loftus EV Jr, Coelho-Prabhu N
Inflamm Bowel Dis 2024 Feb 1;30(2):183-189. doi: 10.1093/ibd/izad021. PMID: 36812365
Is PBC a viral infectious disease?
Mason AL
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:27-39. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.001. PMID: 30343707
Long-term Outcomes and Risk Factors After Adult Living Donor Liver Transplantation.
Imai D, Yoshizumi T, Sakata K, Ikegami T, Itoh S, Harada N, Motomura T, Toshima T, Mano Y, Soejima Y, Maehara Y
Transplantation 2018 Sep;102(9):e382-e391. doi: 10.1097/TP.0000000000002324. PMID: 29912047
Genetic Association of PTPN22 Polymorphisms with Autoimmune Hepatitis and Primary Biliary Cholangitis in Japan.
Umemura T, Joshita S, Yamazaki T, Komatsu M, Katsuyama Y, Yoshizawa K, Tanaka E, Ota M
Sci Rep 2016 Jul 11;6:29770. doi: 10.1038/srep29770. PMID: 27406031Free PMC Article

Prognosis

Mapping the hepatic immune landscape identifies monocytic macrophages as key drivers of steatohepatitis and cholangiopathy progression.
Guillot A, Winkler M, Silva Afonso M, Aggarwal A, Lopez D, Berger H, Kohlhepp MS, Liu H, Özdirik B, Eschrich J, Ma J, Peiseler M, Heymann F, Pendem S, Mahadevan S, Gao B, Diehl L, Gupta R, Tacke F
Hepatology 2023 Jul 1;78(1):150-166. Epub 2023 Jan 13 doi: 10.1097/HEP.0000000000000270. PMID: 36630995
IgG4-related sclerosing cholangitis in the absence of autoimmune pancreatitis mimicking extrahepatic cholangiocarcinoma.
Lin J, Cummings OW, Greenson JK, House MG, Liu X, Nalbantoglu I, Pai R, Davidson DD, Reuss SA
Scand J Gastroenterol 2015 Apr;50(4):447-53. Epub 2015 Jan 30 doi: 10.3109/00365521.2014.962603. PMID: 25635498
The role of natural killer cells in autoimmune liver disease: a comprehensive review.
Hudspeth K, Pontarini E, Tentorio P, Cimino M, Donadon M, Torzilli G, Lugli E, Della Bella S, Gershwin ME, Mavilio D
J Autoimmun 2013 Oct;46:55-65. Epub 2013 Jul 21 doi: 10.1016/j.jaut.2013.07.003. PMID: 23880068
Follicular cholangitis and pancreatitis - clinicopathological features and differential diagnosis of an under-recognized entity.
Zen Y, Ishikawa A, Ogiso S, Heaton N, Portmann B
Histopathology 2012 Jan;60(2):261-9. doi: 10.1111/j.1365-2559.2011.04078.x. PMID: 22211284
IgG4-related sclerosing disease, an emerging entity: a review of a multi-system disease.
Divatia M, Kim SA, Ro JY
Yonsei Med J 2012 Jan;53(1):15-34. doi: 10.3349/ymj.2012.53.1.15. PMID: 22187229Free PMC Article

Clinical prediction guides

Innate lymphoid cell subsets in the pathogenesis of primary biliary cholangitis.
Liu Y, Hu Y, Li B, Su R, Han Z, Jin B, Li T, Zheng X, Han Y
J Gastroenterol Hepatol 2024 Jul;39(7):1431-1441. Epub 2024 Apr 12 doi: 10.1111/jgh.16547. PMID: 38606537
Mapping the hepatic immune landscape identifies monocytic macrophages as key drivers of steatohepatitis and cholangiopathy progression.
Guillot A, Winkler M, Silva Afonso M, Aggarwal A, Lopez D, Berger H, Kohlhepp MS, Liu H, Özdirik B, Eschrich J, Ma J, Peiseler M, Heymann F, Pendem S, Mahadevan S, Gao B, Diehl L, Gupta R, Tacke F
Hepatology 2023 Jul 1;78(1):150-166. Epub 2023 Jan 13 doi: 10.1097/HEP.0000000000000270. PMID: 36630995
Is PBC a viral infectious disease?
Mason AL
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:27-39. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.001. PMID: 30343707
Th1 and Innate Lymphoid Cells Accumulate in Primary Sclerosing Cholangitis-associated Inflammatory Bowel Disease.
Gwela A, Siddhanathi P, Chapman RW, Travis S, Powrie F, Arancibia-Cárcamo CV, Geremia A
J Crohns Colitis 2017 Sep 1;11(9):1124-1134. doi: 10.1093/ecco-jcc/jjx050. PMID: 28383652Free PMC Article
Liver abnormalities in connective tissue diseases.
De Santis M, Crotti C, Selmi C
Best Pract Res Clin Gastroenterol 2013 Aug;27(4):543-51. doi: 10.1016/j.bpg.2013.06.016. PMID: 24090941

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