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Primary biliary cholangitis 1(PBC1)

MedGen UID:
1642459
Concept ID:
C4551595
Disease or Syndrome
Synonym: Primary biliary cirrhosis 1
 
Monarch Initiative: MONDO:0007193
OMIM®: 109720

Clinical features

From HPO
Biliary cirrhosis
MedGen UID:
44182
Concept ID:
C0023892
Disease or Syndrome
Progressive destruction of the small-to-medium bile ducts of the intrahepatic biliary tree, which leads to progressive cholestasis and often end-stage liver disease.

Professional guidelines

PubMed

Brown ZJ, Baghdadi A, Kamel I, Labiner HE, Hewitt DB, Pawlik TM
HPB (Oxford) 2023 Jan;25(1):14-25. Epub 2022 Oct 5 doi: 10.1016/j.hpb.2022.09.010. PMID: 36257874
Eslam M, Newsome PN, Sarin SK, Anstee QM, Targher G, Romero-Gomez M, Zelber-Sagi S, Wai-Sun Wong V, Dufour JF, Schattenberg JM, Kawaguchi T, Arrese M, Valenti L, Shiha G, Tiribelli C, Yki-Järvinen H, Fan JG, Grønbæk H, Yilmaz Y, Cortez-Pinto H, Oliveira CP, Bedossa P, Adams LA, Zheng MH, Fouad Y, Chan WK, Mendez-Sanchez N, Ahn SH, Castera L, Bugianesi E, Ratziu V, George J
J Hepatol 2020 Jul;73(1):202-209. Epub 2020 Apr 8 doi: 10.1016/j.jhep.2020.03.039. PMID: 32278004
Kwo PY, Cohen SM, Lim JK
Am J Gastroenterol 2017 Jan;112(1):18-35. Epub 2016 Dec 20 doi: 10.1038/ajg.2016.517. PMID: 27995906

Recent clinical studies

Etiology

Kowdley KV, Bowlus CL, Levy C, Akarca US, Alvares-da-Silva MR, Andreone P, Arrese M, Corpechot C, Francque SM, Heneghan MA, Invernizzi P, Jones D, Kruger FC, Lawitz E, Mayo MJ, Shiffman ML, Swain MG, Valera JM, Vargas V, Vierling JM, Villamil A, Addy C, Dietrich J, Germain JM, Mazain S, Rafailovic D, Taddé B, Miller B, Shu J, Zein CO, Schattenberg JM; ELATIVE Study Investigators’ Group; ELATIVE Study Investigators' Group
N Engl J Med 2024 Feb 29;390(9):795-805. Epub 2023 Nov 13 doi: 10.1056/NEJMoa2306185. PMID: 37962077
Hirschfield GM, Shiffman ML, Gulamhusein A, Kowdley KV, Vierling JM, Levy C, Kremer AE, Zigmond E, Andreone P, Gordon SC, Bowlus CL, Lawitz EJ, Aspinall RJ, Pratt DS, Raikhelson K, Gonzalez-Huezo MS, Heneghan MA, Jeong SH, Ladrón de Guevara AL, Mayo MJ, Dalekos GN, Drenth JPH, Janczewska E, Leggett BA, Nevens F, Vargas V, Zuckerman E, Corpechot C, Fassio E, Hinrichsen H, Invernizzi P, Trivedi PJ, Forman L, Jones DEJ, Ryder SD, Swain MG, Steinberg A, Boudes PF, Choi YJ, McWherter CA; ENHANCE Study Group*
Hepatology 2023 Aug 1;78(2):397-415. Epub 2023 Apr 6 doi: 10.1097/HEP.0000000000000395. PMID: 37386786Free PMC Article
Colapietro F, Lleo A, Generali E
Clin Rev Allergy Immunol 2022 Oct;63(2):166-177. Epub 2021 Sep 29 doi: 10.1007/s12016-021-08904-y. PMID: 34586589Free PMC Article
Cordell HJ, Fryett JJ, Ueno K, Darlay R, Aiba Y, Hitomi Y, Kawashima M, Nishida N, Khor SS, Gervais O, Kawai Y, Nagasaki M, Tokunaga K, Tang R, Shi Y, Li Z, Juran BD, Atkinson EJ, Gerussi A, Carbone M, Asselta R, Cheung A, de Andrade M, Baras A, Horowitz J, Ferreira MAR, Sun D, Jones DE, Flack S, Spicer A, Mulcahy VL, Byan J, Han Y, Sandford RN, Lazaridis KN, Amos CI, Hirschfield GM, Seldin MF, Invernizzi P, Siminovitch KA, Ma X, Nakamura M, Mells GF; PBC Consortia; Canadian PBC Consortium; Chinese PBC Consortium; Italian PBC Study Group; Japan-PBC-GWAS Consortium; US PBC Consortium; UK-PBC Consortium
J Hepatol 2021 Sep;75(3):572-581. Epub 2021 May 23 doi: 10.1016/j.jhep.2021.04.055. PMID: 34033851Free PMC Article
Younossi ZM, Bernstein D, Shiffman ML, Kwo P, Kim WR, Kowdley KV, Jacobson IM
Am J Gastroenterol 2019 Jan;114(1):48-63. doi: 10.1038/s41395-018-0390-3. PMID: 30429590

Diagnosis

You H, Ma X, Efe C, Wang G, Jeong SH, Abe K, Duan W, Chen S, Kong Y, Zhang D, Wei L, Wang FS, Lin HC, Yang JM, Tanwandee T, Gani RA, Payawal DA, Sharma BC, Hou J, Yokosuka O, Dokmeci AK, Crawford D, Kao JH, Piratvisuth T, Suh DJ, Lesmana LA, Sollano J, Lau G, Sarin SK, Omata M, Tanaka A, Jia J
Hepatol Int 2022 Feb;16(1):1-23. Epub 2022 Feb 4 doi: 10.1007/s12072-021-10276-6. PMID: 35119627Free PMC Article
Colapietro F, Lleo A, Generali E
Clin Rev Allergy Immunol 2022 Oct;63(2):166-177. Epub 2021 Sep 29 doi: 10.1007/s12016-021-08904-y. PMID: 34586589Free PMC Article
Tanaka A
Clin Mol Hepatol 2021 Jan;27(1):1-21. Epub 2020 Dec 3 doi: 10.3350/cmh.2020.0028. PMID: 33264835Free PMC Article
Younossi ZM, Bernstein D, Shiffman ML, Kwo P, Kim WR, Kowdley KV, Jacobson IM
Am J Gastroenterol 2019 Jan;114(1):48-63. doi: 10.1038/s41395-018-0390-3. PMID: 30429590
Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ; American Association for Study of Liver Diseases
Hepatology 2009 Jul;50(1):291-308. doi: 10.1002/hep.22906. PMID: 19554543

Therapy

Kowdley KV, Bowlus CL, Levy C, Akarca US, Alvares-da-Silva MR, Andreone P, Arrese M, Corpechot C, Francque SM, Heneghan MA, Invernizzi P, Jones D, Kruger FC, Lawitz E, Mayo MJ, Shiffman ML, Swain MG, Valera JM, Vargas V, Vierling JM, Villamil A, Addy C, Dietrich J, Germain JM, Mazain S, Rafailovic D, Taddé B, Miller B, Shu J, Zein CO, Schattenberg JM; ELATIVE Study Investigators’ Group; ELATIVE Study Investigators' Group
N Engl J Med 2024 Feb 29;390(9):795-805. Epub 2023 Nov 13 doi: 10.1056/NEJMoa2306185. PMID: 37962077
Hirschfield GM, Shiffman ML, Gulamhusein A, Kowdley KV, Vierling JM, Levy C, Kremer AE, Zigmond E, Andreone P, Gordon SC, Bowlus CL, Lawitz EJ, Aspinall RJ, Pratt DS, Raikhelson K, Gonzalez-Huezo MS, Heneghan MA, Jeong SH, Ladrón de Guevara AL, Mayo MJ, Dalekos GN, Drenth JPH, Janczewska E, Leggett BA, Nevens F, Vargas V, Zuckerman E, Corpechot C, Fassio E, Hinrichsen H, Invernizzi P, Trivedi PJ, Forman L, Jones DEJ, Ryder SD, Swain MG, Steinberg A, Boudes PF, Choi YJ, McWherter CA; ENHANCE Study Group*
Hepatology 2023 Aug 1;78(2):397-415. Epub 2023 Apr 6 doi: 10.1097/HEP.0000000000000395. PMID: 37386786Free PMC Article
Schattenberg JM, Pares A, Kowdley KV, Heneghan MA, Caldwell S, Pratt D, Bonder A, Hirschfield GM, Levy C, Vierling J, Jones D, Tailleux A, Staels B, Megnien S, Hanf R, Magrez D, Birman P, Luketic V
J Hepatol 2021 Jun;74(6):1344-1354. Epub 2021 Jan 21 doi: 10.1016/j.jhep.2021.01.013. PMID: 33484775
Lleo A, Leung PSC, Hirschfield GM, Gershwin EM
Semin Liver Dis 2020 Feb;40(1):34-48. Epub 2019 Sep 19 doi: 10.1055/s-0039-1697617. PMID: 31537031
Nevens F, Andreone P, Mazzella G, Strasser SI, Bowlus C, Invernizzi P, Drenth JP, Pockros PJ, Regula J, Beuers U, Trauner M, Jones DE, Floreani A, Hohenester S, Luketic V, Shiffman M, van Erpecum KJ, Vargas V, Vincent C, Hirschfield GM, Shah H, Hansen B, Lindor KD, Marschall HU, Kowdley KV, Hooshmand-Rad R, Marmon T, Sheeron S, Pencek R, MacConell L, Pruzanski M, Shapiro D; POISE Study Group
N Engl J Med 2016 Aug 18;375(7):631-43. doi: 10.1056/NEJMoa1509840. PMID: 27532829

Prognosis

Corpechot C, Carrat F, Gaouar F, Chau F, Hirschfield G, Gulamhusein A, Montano-Loza AJ, Lytvyak E, Schramm C, Pares A, Olivas I, Eaton JE, Osman KT, Dalekos G, Gatselis N, Nevens F, Cazzagon N, Zago A, Russo FP, Abbas N, Trivedi P, Thorburn D, Saffioti F, Barkai L, Roccarina D, Calvaruso V, Fichera A, Delamarre A, Medina-Morales E, Bonder A, Patwardhan V, Rigamonti C, Carbone M, Invernizzi P, Cristoferi L, van der Meer A, de Veer R, Zigmond E, Yehezkel E, Kremer AE, Deibel A, Dumortier J, Bruns T, Große K, Pageaux GP, Wetten A, Dyson J, Jones D, Chazouillères O, Hansen B, de Lédinghen V; Global & ERN Rare-Liver PBC Study Groups
J Hepatol 2022 Dec;77(6):1545-1553. Epub 2022 Jun 28 doi: 10.1016/j.jhep.2022.06.017. PMID: 35777587
Trivedi PJ, Hirschfield GM
Gut 2021 Oct;70(10):1989-2003. Epub 2021 Jul 15 doi: 10.1136/gutjnl-2020-322362. PMID: 34266966
Cordell HJ, Fryett JJ, Ueno K, Darlay R, Aiba Y, Hitomi Y, Kawashima M, Nishida N, Khor SS, Gervais O, Kawai Y, Nagasaki M, Tokunaga K, Tang R, Shi Y, Li Z, Juran BD, Atkinson EJ, Gerussi A, Carbone M, Asselta R, Cheung A, de Andrade M, Baras A, Horowitz J, Ferreira MAR, Sun D, Jones DE, Flack S, Spicer A, Mulcahy VL, Byan J, Han Y, Sandford RN, Lazaridis KN, Amos CI, Hirschfield GM, Seldin MF, Invernizzi P, Siminovitch KA, Ma X, Nakamura M, Mells GF; PBC Consortia; Canadian PBC Consortium; Chinese PBC Consortium; Italian PBC Study Group; Japan-PBC-GWAS Consortium; US PBC Consortium; UK-PBC Consortium
J Hepatol 2021 Sep;75(3):572-581. Epub 2021 May 23 doi: 10.1016/j.jhep.2021.04.055. PMID: 34033851Free PMC Article
Tanaka A
Clin Mol Hepatol 2021 Jan;27(1):1-21. Epub 2020 Dec 3 doi: 10.3350/cmh.2020.0028. PMID: 33264835Free PMC Article
Younossi ZM, Bernstein D, Shiffman ML, Kwo P, Kim WR, Kowdley KV, Jacobson IM
Am J Gastroenterol 2019 Jan;114(1):48-63. doi: 10.1038/s41395-018-0390-3. PMID: 30429590

Clinical prediction guides

Kowdley KV, Bowlus CL, Levy C, Akarca US, Alvares-da-Silva MR, Andreone P, Arrese M, Corpechot C, Francque SM, Heneghan MA, Invernizzi P, Jones D, Kruger FC, Lawitz E, Mayo MJ, Shiffman ML, Swain MG, Valera JM, Vargas V, Vierling JM, Villamil A, Addy C, Dietrich J, Germain JM, Mazain S, Rafailovic D, Taddé B, Miller B, Shu J, Zein CO, Schattenberg JM; ELATIVE Study Investigators’ Group; ELATIVE Study Investigators' Group
N Engl J Med 2024 Feb 29;390(9):795-805. Epub 2023 Nov 13 doi: 10.1056/NEJMoa2306185. PMID: 37962077
Hirschfield GM, Shiffman ML, Gulamhusein A, Kowdley KV, Vierling JM, Levy C, Kremer AE, Zigmond E, Andreone P, Gordon SC, Bowlus CL, Lawitz EJ, Aspinall RJ, Pratt DS, Raikhelson K, Gonzalez-Huezo MS, Heneghan MA, Jeong SH, Ladrón de Guevara AL, Mayo MJ, Dalekos GN, Drenth JPH, Janczewska E, Leggett BA, Nevens F, Vargas V, Zuckerman E, Corpechot C, Fassio E, Hinrichsen H, Invernizzi P, Trivedi PJ, Forman L, Jones DEJ, Ryder SD, Swain MG, Steinberg A, Boudes PF, Choi YJ, McWherter CA; ENHANCE Study Group*
Hepatology 2023 Aug 1;78(2):397-415. Epub 2023 Apr 6 doi: 10.1097/HEP.0000000000000395. PMID: 37386786Free PMC Article
Trivedi PJ, Hirschfield GM
Gut 2021 Oct;70(10):1989-2003. Epub 2021 Jul 15 doi: 10.1136/gutjnl-2020-322362. PMID: 34266966
Cordell HJ, Fryett JJ, Ueno K, Darlay R, Aiba Y, Hitomi Y, Kawashima M, Nishida N, Khor SS, Gervais O, Kawai Y, Nagasaki M, Tokunaga K, Tang R, Shi Y, Li Z, Juran BD, Atkinson EJ, Gerussi A, Carbone M, Asselta R, Cheung A, de Andrade M, Baras A, Horowitz J, Ferreira MAR, Sun D, Jones DE, Flack S, Spicer A, Mulcahy VL, Byan J, Han Y, Sandford RN, Lazaridis KN, Amos CI, Hirschfield GM, Seldin MF, Invernizzi P, Siminovitch KA, Ma X, Nakamura M, Mells GF; PBC Consortia; Canadian PBC Consortium; Chinese PBC Consortium; Italian PBC Study Group; Japan-PBC-GWAS Consortium; US PBC Consortium; UK-PBC Consortium
J Hepatol 2021 Sep;75(3):572-581. Epub 2021 May 23 doi: 10.1016/j.jhep.2021.04.055. PMID: 34033851Free PMC Article
Hirschfield GM, Dyson JK, Alexander GJM, Chapman MH, Collier J, Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DEJ
Gut 2018 Sep;67(9):1568-1594. Epub 2018 Mar 28 doi: 10.1136/gutjnl-2017-315259. PMID: 29593060Free PMC Article

Recent systematic reviews

Orci LA, Sanduzzi-Zamparelli M, Caballol B, Sapena V, Colucci N, Torres F, Bruix J, Reig M, Toso C
Clin Gastroenterol Hepatol 2022 Feb;20(2):283-292.e10. Epub 2021 May 28 doi: 10.1016/j.cgh.2021.05.002. PMID: 33965578
Cordell HJ, Fryett JJ, Ueno K, Darlay R, Aiba Y, Hitomi Y, Kawashima M, Nishida N, Khor SS, Gervais O, Kawai Y, Nagasaki M, Tokunaga K, Tang R, Shi Y, Li Z, Juran BD, Atkinson EJ, Gerussi A, Carbone M, Asselta R, Cheung A, de Andrade M, Baras A, Horowitz J, Ferreira MAR, Sun D, Jones DE, Flack S, Spicer A, Mulcahy VL, Byan J, Han Y, Sandford RN, Lazaridis KN, Amos CI, Hirschfield GM, Seldin MF, Invernizzi P, Siminovitch KA, Ma X, Nakamura M, Mells GF; PBC Consortia; Canadian PBC Consortium; Chinese PBC Consortium; Italian PBC Study Group; Japan-PBC-GWAS Consortium; US PBC Consortium; UK-PBC Consortium
J Hepatol 2021 Sep;75(3):572-581. Epub 2021 May 23 doi: 10.1016/j.jhep.2021.04.055. PMID: 34033851Free PMC Article
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
Schreuder AM, Busch OR, Besselink MG, Ignatavicius P, Gulbinas A, Barauskas G, Gouma DJ, van Gulik TM
Dig Surg 2020;37(1):10-21. Epub 2019 Jan 17 doi: 10.1159/000496432. PMID: 30654363Free PMC Article
Zacharias AP, Jeyaraj R, Hobolth L, Bendtsen F, Gluud LL, Morgan MY
Cochrane Database Syst Rev 2018 Oct 29;10(10):CD011510. doi: 10.1002/14651858.CD011510.pub2. PMID: 30372514Free PMC Article

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