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Primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome

MedGen UID:
1812237
•Concept ID:
C5680117
•
Disease or Syndrome
Synonyms: Overlap syndrome of autoimmune liver disease; Overlap syndromes of autoimmune liver diseases; PBC/PSC and AIH overlap syndrome; Primary biliary cholangitis and/or primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome; primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome
SNOMED CT: Primary biliary cholangitis and/or primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome (1230291009); Overlap syndrome of autoimmune liver disease (1230291009)
 
Monarch Initiative: MONDO:0034189
Orphanet: ORPHA562639

Definition

A rare hepatic disease characterized by the overlap of primary biliary cholangitis and/or primary sclerosing cholangitis with autoimmune hepatitis, defined by the presence of at least two of the three recognized biochemical, serological and histological criteria of each disease. The onset of the overlapping diseases can be simultaneous or sequential with a variable interval of up to several years. Age of onset, gender predisposition and clinical phenotype vary between each of the diseases. The clinical presentation ranges from asymptomatic disease or unspecific symptoms such as fatigue, arthralgia and pruritus to established cirrhosis and decompensation or also acute fulminant hepatitis and liver failure. Association with extrahepatic autoimmune diseases is common. [from SNOMEDCT_US]

Professional guidelines

PubMed

Non-invasive diagnosis and follow-up of autoimmune hepatitis.
Lemoinne S, Heurgue A, Bouzbib C, Hanslik B, Gournay J, Nguyen-Khac E, Bureau C, de Lédinghen V, Ganne-Carrié N, Bourlière M
Clin Res Hepatol Gastroenterol 2022 Jan;46(1):101772. Epub 2021 Jul 28 doi: 10.1016/j.clinre.2021.101772. PMID: 34332126
Autoimmune Hepatitis and Overlap Syndromes: Diagnosis and Management.
Vierling JM
Clin Gastroenterol Hepatol 2015 Nov;13(12):2088-108. Epub 2015 Aug 15 doi: 10.1016/j.cgh.2015.08.012. PMID: 26284592
Diagnosis and management of overlap syndromes.
Bunchorntavakul C, Reddy KR
Clin Liver Dis 2015 Feb;19(1):81-97. Epub 2014 Nov 21 doi: 10.1016/j.cld.2014.09.005. PMID: 25454298

Recent clinical studies

Etiology

Autoimmune liver diseases and diabetes: A propensity score matched analysis and a proportional meta-analysis.
Jensen AH, Winther-Sørensen M, Burisch J, Bergquist A, Ytting H, Gluud LL, Wewer Albrechtsen NJ
Liver Int 2023 Nov;43(11):2479-2491. Epub 2023 Sep 26 doi: 10.1111/liv.15720. PMID: 37752719
Non-invasive diagnosis and follow-up of autoimmune hepatitis.
Lemoinne S, Heurgue A, Bouzbib C, Hanslik B, Gournay J, Nguyen-Khac E, Bureau C, de Lédinghen V, Ganne-Carrié N, Bourlière M
Clin Res Hepatol Gastroenterol 2022 Jan;46(1):101772. Epub 2021 Jul 28 doi: 10.1016/j.clinre.2021.101772. PMID: 34332126
Imaging of autoimmune hepatitis and overlap syndromes.
Malik N, Venkatesh SK
Abdom Radiol (NY) 2017 Jan;42(1):19-27. doi: 10.1007/s00261-016-1019-x. PMID: 27999888
Overlap Syndromes.
Chazouillères O
Dig Dis 2015;33 Suppl 2:181-7. Epub 2015 Dec 7 doi: 10.1159/000440831. PMID: 26641819
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

Diagnosis

Variant syndromes of primary biliary cholangitis.
Schulz L, Sebode M, Weidemann SA, Lohse AW
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:55-61. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.003. PMID: 30343711
Overlap Syndromes.
Chazouillères O
Dig Dis 2015;33 Suppl 2:181-7. Epub 2015 Dec 7 doi: 10.1159/000440831. PMID: 26641819
Autoimmune Hepatitis and Overlap Syndromes: Diagnosis and Management.
Vierling JM
Clin Gastroenterol Hepatol 2015 Nov;13(12):2088-108. Epub 2015 Aug 15 doi: 10.1016/j.cgh.2015.08.012. PMID: 26284592
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
Overlap syndromes.
Beuers U, Rust C
Semin Liver Dis 2005 Aug;25(3):311-20. doi: 10.1055/s-2005-916322. PMID: 16143946

Therapy

Autoimmune liver diseases and diabetes: A propensity score matched analysis and a proportional meta-analysis.
Jensen AH, Winther-Sørensen M, Burisch J, Bergquist A, Ytting H, Gluud LL, Wewer Albrechtsen NJ
Liver Int 2023 Nov;43(11):2479-2491. Epub 2023 Sep 26 doi: 10.1111/liv.15720. PMID: 37752719
Variant syndromes of primary biliary cholangitis.
Schulz L, Sebode M, Weidemann SA, Lohse AW
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:55-61. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.003. PMID: 30343711
Overlap Syndromes.
Chazouillères O
Dig Dis 2015;33 Suppl 2:181-7. Epub 2015 Dec 7 doi: 10.1159/000440831. PMID: 26641819
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
Overlap syndromes.
Beuers U, Rust C
Semin Liver Dis 2005 Aug;25(3):311-20. doi: 10.1055/s-2005-916322. PMID: 16143946

Prognosis

Non-invasive diagnosis and follow-up of autoimmune hepatitis.
Lemoinne S, Heurgue A, Bouzbib C, Hanslik B, Gournay J, Nguyen-Khac E, Bureau C, de Lédinghen V, Ganne-Carrié N, Bourlière M
Clin Res Hepatol Gastroenterol 2022 Jan;46(1):101772. Epub 2021 Jul 28 doi: 10.1016/j.clinre.2021.101772. PMID: 34332126
Variant syndromes of primary biliary cholangitis.
Schulz L, Sebode M, Weidemann SA, Lohse AW
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:55-61. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.003. PMID: 30343711
Autoimmune Hepatitis and Overlap Syndromes: Diagnosis and Management.
Vierling JM
Clin Gastroenterol Hepatol 2015 Nov;13(12):2088-108. Epub 2015 Aug 15 doi: 10.1016/j.cgh.2015.08.012. PMID: 26284592
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
Overlap syndromes.
Beuers U, Rust C
Semin Liver Dis 2005 Aug;25(3):311-20. doi: 10.1055/s-2005-916322. PMID: 16143946

Clinical prediction guides

Autoimmune liver diseases and diabetes: A propensity score matched analysis and a proportional meta-analysis.
Jensen AH, Winther-Sørensen M, Burisch J, Bergquist A, Ytting H, Gluud LL, Wewer Albrechtsen NJ
Liver Int 2023 Nov;43(11):2479-2491. Epub 2023 Sep 26 doi: 10.1111/liv.15720. PMID: 37752719
Clinical aspects and prognosis of patients with inflammatory bowel disease associated with autoimmune liver diseases.
Rocha HC, Vilela EG
Gastroenterol Hepatol 2022 Feb;45(2):83-90. Epub 2021 May 20 doi: 10.1016/j.gastrohep.2021.03.011. PMID: 34023469
Variant syndromes of primary biliary cholangitis.
Schulz L, Sebode M, Weidemann SA, Lohse AW
Best Pract Res Clin Gastroenterol 2018 Jun-Aug;34-35:55-61. Epub 2018 Jun 14 doi: 10.1016/j.bpg.2018.06.003. PMID: 30343711
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
The overlap syndromes of autoimmune hepatitis.
Czaja AJ
Dig Dis Sci 2013 Feb;58(2):326-43. Epub 2012 Aug 24 doi: 10.1007/s10620-012-2367-1. PMID: 22918690

Recent systematic reviews

Autoimmune liver diseases and diabetes: A propensity score matched analysis and a proportional meta-analysis.
Jensen AH, Winther-Sørensen M, Burisch J, Bergquist A, Ytting H, Gluud LL, Wewer Albrechtsen NJ
Liver Int 2023 Nov;43(11):2479-2491. Epub 2023 Sep 26 doi: 10.1111/liv.15720. PMID: 37752719
Skin Manifestations Associated with Autoimmune Liver Diseases: a Systematic Review.
Terziroli Beretta-Piccoli B, Invernizzi P, Gershwin ME, Mainetti C
Clin Rev Allergy Immunol 2017 Dec;53(3):394-412. doi: 10.1007/s12016-017-8649-9. PMID: 28993983
Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver.
Bittencourt PL, Cançado EL, Couto CA, Levy C, Porta G, Silva AE, Terrabuio DR; Brazilian Society of Hepatology on the Diagnosis and Management of Autoimmune Diseases of the Liver, Carvalho Filho RJ, Chaves DM, Miura IK, Codes L, Faria LC, Evangelista AS, Farias AQ, Gonçalves LL, Harriz M, Lopes Neto EP, Luz GO, Oliveira P, Oliveira EM, Schiavon JL, Seva-Pereira T, Parise ER
Arq Gastroenterol 2015 Dec;52 Suppl 1:15-46. doi: 10.1590/S0004-28032015000500002. PMID: 26959804
Systematic review: management options for primary sclerosing cholangitis and its variant forms - IgG4-associated cholangitis and overlap with autoimmune hepatitis.
Culver EL, Chapman RW
Aliment Pharmacol Ther 2011 Jun;33(12):1273-91. Epub 2011 Apr 18 doi: 10.1111/j.1365-2036.2011.04658.x. PMID: 21501198

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