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Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis.
Sarcognato S, Sacchi D, Grillo F, Cazzagon N, Fabris L, Cadamuro M, Cataldo I, Covelli C, Mangia A, Guido M. Sarcognato S, et al. Pathologica. 2021 Jun;113(3):170-184. doi: 10.32074/1591-951X-245. Pathologica. 2021. PMID: 34294935 Free PMC article. Review.
Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic live …
Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile duc …
Primary biliary cholangitis.
Parés A. Parés A. Med Clin (Barc). 2018 Sep 21;151(6):242-249. doi: 10.1016/j.medcli.2017.12.021. Epub 2018 Mar 9. Med Clin (Barc). 2018. PMID: 29526309 Review. English, Spanish.
Ursodeoxycholic acid is the specific treatment with an excellent response in more than 60% of patients. When this optimal response is not observed, it can be combined with new agents, but those that have shown to be effective are those that improve cholestasis such as fibr …
Ursodeoxycholic acid is the specific treatment with an excellent response in more than 60% of patients. When this optimal response is not …
Efficacy and Safety of Elafibranor in Primary Biliary Cholangitis.
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. Kowdley KV, et al. N Engl J Med. 2024 Feb 29;390(9):795-805. doi: 10.1056/NEJMoa2306185. Epub 2023 Nov 13. N Engl J Med. 2024. PMID: 37962077 Clinical Trial.
BACKGROUND: Primary biliary cholangitis is a rare, chronic cholestatic liver disease characterized by the destruction of interlobular bile ducts, leading to cholestasis and liver fibrosis. Whether elafibranor, an oral, dual peroxisome proliferator-activated r …
BACKGROUND: Primary biliary cholangitis is a rare, chronic cholestatic liver disease characterized by the destruction o …
P4HA2 induces hepatic ductular reaction and biliary fibrosis in chronic cholestatic liver diseases.
Zhang J, Lyu Z, Li B, You Z, Cui N, Li Y, Li Y, Huang B, Chen R, Chen Y, Peng Y, Fang J, Wang Q, Miao Q, Tang R, Gershwin ME, Lian M, Xiao X, Ma X. Zhang J, et al. Hepatology. 2023 Jul 1;78(1):10-25. doi: 10.1097/HEP.0000000000000317. Epub 2023 Feb 20. Hepatology. 2023. PMID: 36799463
We postulated that P4HA2 would play a key role in the cholangiocyte pathology of cholestatic liver diseases. METHODS: We studied humans with primary biliary cholangitis (PBC) and Primary sclerosing cholangitis (PSC), P4HA2 -/- mice injured by DDC, and P4HA2 - …
We postulated that P4HA2 would play a key role in the cholangiocyte pathology of cholestatic liver diseases. METHODS: We studied humans with …
Recent advances in clinical practice: epidemiology of autoimmune liver diseases.
Trivedi PJ, Hirschfield GM. Trivedi PJ, et al. Gut. 2021 Oct;70(10):1989-2003. doi: 10.1136/gutjnl-2020-322362. Epub 2021 Jul 15. Gut. 2021. PMID: 34266966 Review.
Autoimmune liver diseases are chronic inflammatory hepatobiliary disorders that when classically defined encompass three distinctive clinical presentations; primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) …
Autoimmune liver diseases are chronic inflammatory hepatobiliary disorders that when classically defined encompass three distinctive clinica …
A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis.
Corpechot C, Chazouillères O, Rousseau A, Le Gruyer A, Habersetzer F, Mathurin P, Goria O, Potier P, Minello A, Silvain C, Abergel A, Debette-Gratien M, Larrey D, Roux O, Bronowicki JP, Boursier J, de Ledinghen V, Heurgue-Berlot A, Nguyen-Khac E, Zoulim F, Ollivier-Hourmand I, Zarski JP, Nkontchou G, Lemoinne S, Humbert L, Rainteau D, Lefèvre G, de Chaisemartin L, Chollet-Martin S, Gaouar F, Admane FH, Simon T, Poupon R. Corpechot C, et al. N Engl J Med. 2018 Jun 7;378(23):2171-2181. doi: 10.1056/NEJMoa1714519. N Engl J Med. 2018. PMID: 29874528 Free article. Clinical Trial.
BACKGROUND: Patients with primary biliary cholangitis who have an inadequate response to therapy with ursodeoxycholic acid are at high risk for disease progression. ...Myalgia occurred in 20% of the patients in the bezafibrate group and in 10% in the placebo …
BACKGROUND: Patients with primary biliary cholangitis who have an inadequate response to therapy with ursodeoxycholic a …
Seladelpar efficacy and safety at 3 months in patients with primary biliary cholangitis: ENHANCE, a phase 3, randomized, placebo-controlled study.
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*. Hirschfield GM, et al. Hepatology. 2023 Aug 1;78(2):397-415. doi: 10.1097/HEP.0000000000000395. Epub 2023 Apr 6. Hepatology. 2023. PMID: 37386786 Free PMC article. Clinical Trial.
BACKGROUND AND AIMS: ENHANCE was a phase 3 study that evaluated efficacy and safety of seladelpar, a selective peroxisome proliferator-activated receptor-delta (PPAR) agonist, versus placebo in patients with primary biliary cholangitis with inadequate respons …
BACKGROUND AND AIMS: ENHANCE was a phase 3 study that evaluated efficacy and safety of seladelpar, a selective peroxisome proliferator-activ …
The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines.
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. Hirschfield GM, et al. Gut. 2018 Sep;67(9):1568-1594. doi: 10.1136/gutjnl-2017-315259. Epub 2018 Mar 28. Gut. 2018. PMID: 29593060 Free PMC article.
Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage bili …
Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which …
Liver stiffness measurement by vibration-controlled transient elastography improves outcome prediction in primary biliary cholangitis.
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. Corpechot C, et al. J Hepatol. 2022 Dec;77(6):1545-1553. doi: 10.1016/j.jhep.2022.06.017. Epub 2022 Jun 28. J Hepatol. 2022. PMID: 35777587 Free article.
BACKGROUND & AIMS: Liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) has been shown to predict outcomes of patients with primary biliary cholangitis (PBC) in small-size studies. ...LAY SUMMARY: Primary …
BACKGROUND & AIMS: Liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) has been shown to predict …
A Phase 3 Trial of Seladelpar in Primary Biliary Cholangitis.
Hirschfield GM, Bowlus CL, Mayo MJ, Kremer AE, Vierling JM, Kowdley KV, Levy C, Villamil A, Ladrón de Guevara Cetina AL, Janczewska E, Zigmond E, Jeong SH, Yilmaz Y, Kallis Y, Corpechot C, Buggisch P, Invernizzi P, Londoño Hurtado MC, Bergheanu S, Yang K, Choi YJ, Crittenden DB, McWherter CA; RESPONSE Study Group. Hirschfield GM, et al. N Engl J Med. 2024 Feb 29;390(9):783-794. doi: 10.1056/NEJMoa2312100. Epub 2024 Feb 21. N Engl J Med. 2024. PMID: 38381664 Clinical Trial.
BACKGROUND: Effective treatments for patients with primary biliary cholangitis are limited. Seladelpar, a peroxisome proliferator-activated receptor delta agonist, has potential benefits. ...Adverse events were reported in 86.7% of the patients in the seladel …
BACKGROUND: Effective treatments for patients with primary biliary cholangitis are limited. Seladelpar, a peroxisome pr …
471 results