Year 2019 / Volume 111 / Number 1
Review
Risk stratification and treatment of primary biliary cholangitis

63-70

DOI: 10.17235/reed.2018.5662/2018

Javier Martínez, Lara Aguilera, Agustín Albillos,

Abstract
Primary biliary cholangitis is a chronic liver disorder characterized by progressive cholestasis that may evolve to liver cirrhosis. While ursodeoxycholic acid is the treatment of choice, around 30% of patients do not respond to this therapy. These patients have a poorer prognosis, hence should be identified early in order to be offered therapy options. Along these lines, improved understanding of the condition's pathophysiology has allowed the development of newer drugs, including obeticholic acid and fibrates. This review offers a perspective on risk stratification and treatment for these patients, from ursodeoxycholic acid to second-line treatments.
Share Button
New comment
Comments

30/05/2020 1:20:46
De mucho interés para la comunidad de Gastroenterologia


References
1.Murillo F, Goet JC, Lammers WJ, et al. from GLOBAL PBC Study Group. Milder disease stage in patients with primary biliary cholangitis over a 44-year period: A changing natural history. Hepatology 2017 [in press].
2.Webb GJ, Rana A, Hodson J, et al. Twenty-Year Comparative Analysis of Patients With Autoimmune Liver Diseases on Transplant Waitlists. Clin Gastroenterol Hepatol 2018; 16:278-287.
3.Beuers U, Gershwin ME, Gish RG, et al. Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis'. J Hepatol 2015; 63:1285-7.
4.Poupon R. Primary biliary cirrhosis: a 2010 update. J Hepatol 2010; 52:745–58.
5.Hirschfield GM. Diagnosis of primary biliary cirrhosis. Best Pract Res Clin Gastroenterol 2011; 25:701–12.
6.Hirschfield GM, Beuers U, Corpechot C, et al. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017; 67:145-172.
7.Carbone M, Mells GF, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterol 2013; 144:560-9.
8.Corpechot C. Utility of Noninvasive Markers of Fibrosis in Cholestatic Liver Diseases. Clin Liver Dis 2016; 20:143-58.
9.Corpechot C, Carrat F, Poujol-Robert A, et al. Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis. Hepatol 2012; 56:198-208.
10.ter Borg PC, Schalm SW, Hansen BE, et al. Prognosis of ursodeoxycholic Acid-treated patients with primary biliary cirrhosis. Results of a 10-yr cohort study involving 297 patients. Am J Gastroenterol 2006; 101:2044-50.
11.Trivedi PJ, Corpechot C, Pares A, et al. Risk stratification in autoimmune cholestatic liver diseases: Opportunities for clinicians and trialists. Hepatol 2016; 63:644-59.
12.Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterol 2006; 130:715-20.
13.Lammers WJ, van Buuren HR, Hirschfield GM, et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterol 2014; 147:1338–49.
14.Zhang L-N, Shi T-Y, Shi X-H, et al. Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: results of a 14-year cohort study. Hepatol 2013; 58:264–72.
15.Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatol 2008; 48:871–7.
16.Silveira MG, Brunt EM, Heathcote J, et al. American Association for the Study of Liver Diseases endpoints conference: design and endpoints for clinical trials in primary biliary cirrhosis. Hepatol 2010; 52:349–59.
17.Trivedi PJ, Bruns T, Cheung A, et al. Optimising risk stratification in primary biliary cirrhosis: AST/platelet ratio index predicts outcome independent of ursodeoxycholic acid response. J Hepatol 2014; 60:1249–58.
18.Harms MH, Lammers WJ, Thorburn D, et al. Major Hepatic Complications in Ursodeoxycholic Acid-Treated Patients With Primary Biliary Cholangitis: Risk Factors and Time Trends in Incidence and Outcome. Am J Gastroenterol 2018; 113:254-264.
19.Corpechot C, Chazouillères O, Poupon R. Early primary biliary cirrhosis: biochemical response to treatment and prediction of long-term outcome. J Hepatol 2011; 55:1361-7.
20.Carbone M, Sharp SJ, Flack S, et al. The UK-PBC risk scores: Derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatol 2016; 63:930–50.
21.Lammers WJ, Hirschfield GM, Corpechot C, et al. Development and Validation of a Scoring System to Predict Outcomes of Patients With Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy. Gastroenterol 2015; 149:1804-12.
22.Quarneti C, Muratori P, Lalanne C. Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis. Liver Int 2015; 35:636-41.
23.Jones DE, Al-Rifai A, Frith J, et al. The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up. J Hepatol 2010; 53:911-7.
24.Rigopoulou EI, Davies ET, Pares A, et al. Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis. Gut 2005; 54:528-32.
25.Wesierska-Gadek J, Penner E, Battezzati PM, et al. Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis. Hepatol 2006; 43:1135-44.
26.Hofmann AF. Pharmacology of ursodeoxycholic acid, an enterohepatic drug. Scand J Gastroenterol Suppl 1994; 204:1–15.
27.Pusl T, Beuers U. Ursodeoxycholic acid treatment of vanishing bile duct syndromes. World J Gastroenterol 2006; 12:3487–95.
28.Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid “mechanisms of action and clinical use in hepatobiliary disorders.” J Hepatol 2001; 35:134–46.
29.Roma MG, Toledo FD, Boaglio AC, et al. Ursodeoxycholic acid in cholestasis: linking action mechanisms to therapeutic applications. Clin Sci 2011; 121:523–44.
30.Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatol 2009; 50:291–308.
31.Poupon R, Chrétien Y, Poupon RE, et al. Is ursodeoxycholic acid an effective treatment for primary biliary cirrhosis? Lancet 1987; 1:834–6.
32.Parés A, Caballería L, Rodés J, et al. Long-term effects of ursodeoxycholic acid in primary biliary cirrhosis: results of a double-blind controlled multicentric trial. UDCA-Cooperative Group from the Spanish Association for the Study of the Liver. J Hepatol 2000; 32:561–6.
33.Heathcote EJ, Cauch-Dudek K, Walker V, et al. The Canadian Multicenter Double-blind Randomized Controlled Trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatol 1994; 19:1149–56.
34.Combes B, Carithers RL, Maddrey WC, et al. A randomized, double-blind, placebo-controlled trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatol 1995; 22:759-66.
35.Corpechot C, Carrat F, Bonnand AM, et al. The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatol 2000; 32:1196–9.
36.Poupon RE, Lindor KD, Parés A, et al. Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis. J Hepatol 2003; 39:12–6.
37.Goulis J, Leandro G, Burroughs AK. Randomised controlled trials of ursodeoxycholic-acid therapy for primary biliary cirrhosis: a meta-analysis. Lancet 1999; 354:1053–60.
38.Gong Y, Huang ZB, Christensen E, et al. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database Syst Rev. 2008; 3:CD000551.
39.Rudic JS, Poropat G, Krstic MN, et al. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database Syst Rev. 2012;12:CD000551.
40.Shi J, Wu C, Lin Y, et al. Long-term effects of mid-dose ursodeoxycholic acid in primary biliary cirrhosis: a meta-analysis of randomized controlled trials. Am J Gastroenterol 2006; 101: 1529-38.
41.Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterol 1997; 113:884–90.
42.Lee J, Belanger A, Doucette JT, et al. Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007; 5:1313-5.
43.Kuiper EMM, Hansen BE, Metselaar HJ, et al. Trends in liver transplantation for primary biliary cirrhosis in the Netherlands 1988-2008. BMC Gastroenterol 2010; 10:144.
44.Pasha T, Heathcote J, Gabriel S, et al. Cost effectiveness of ursodeoxycholic acid therapy in primary biliary cirrhosis. Hepatol 1999; 29:21-6.
45.Boberg KM, Wisløff T, Kjøllesdal KS, et al. Cost and health consequences of treatment of primary biliary cirrhosis with ursodeoxycholic acid. Aliment Pharmacol Ther 2013; 38:794–803.
46.Angulo P, Dickson ER, Therneau TM, et al. Comparison of three doses of ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a randomized trial. J Hepatol 1999; 30:830-5.
47.Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. N Engl J Med 2007; 357:1524–9.
48.Sauer P, Benz C, Rudolph G, et al. Influence of cholestasis on absorption of ursodeoxycholic acid. Dig Dis Sci 1999; 44:817-22.
49.Angulo P, Jorgensen RA, Lindor KD. Incomplete response to ursodeoxycholic acid in primary biliary cirrhosis: is a double dosage worthwhile? Am J Gastroenterol 2001; 96:3152-7.
50.Siegel JL, Jorgensen R, Angulo P, et al. Treatment with ursodeoxycholic acid is associated with weight gain in patients with primary biliary cirrhosis. J Clin Gastroenterol 2003; 37:183-5.
Related articles

Editorial

Treatment of portal vein thrombosis in cirrhosis patients

DOI: 10.17235/reed.2023.9800/2023

Editorial

AKI-HRS, more than a name change for type-1 hepatorenal syndrome

DOI: 10.17235/reed.2023.9677/2023

Letter

Lymphocytic colitis with macroscopic findings

DOI: 10.17235/reed.2023.9497/2023

Editorial

New actors come into play against hepatitis delta

DOI: 10.17235/reed.2022.9050/2022

Letter

Hemoperitoneum due to rupture of intra-abdominal varices

DOI: 10.17235/reed.2022.8937/2022

Letter

Cirrhotic pattern in advanced hepatic sarcoidosis

DOI: 10.17235/reed.2021.8446/2021

Digestive Diseases Image

Giant cystic hepatocarcinoma in the absence of cirrhosis

DOI: 10.17235/reed.2021.8313/2021

Review

Practical management of primary biliary cholangitis

DOI: 10.17235/reed.2021.8219/2021

Review

Physical frailty in liver transplantation

DOI: 10.17235/reed.2020.7448/2020

Citation tools
Martínez J, Aguilera L, Albillos A. Risk stratification and treatment of primary biliary cholangitis. 5662/2018


Download to a citation manager

Download the citation for this article by clicking on one of the following citation managers:

Metrics
This article has received 1631 visits.
This article has been downloaded 955 times.

Statistics from Dimensions


Statistics from Plum Analytics

Publication history

Received: 18/04/2018

Accepted: 11/09/2018

Online First: 19/10/2018

Published: 17/01/2019

Article revision time: 139 days

Article Online First time: 184 days

Article editing time: 274 days


Share
This article hasn't been rated yet.
Reader rating:
Valora este artículo:




Asociación Española de Ecografía Digestiva Sociedad Española de Endoscopia Digestiva Sociedad Española de Patología Digestiva
The Spanish Journal of Gastroenterology is the official organ of the Sociedad Española de Patología Digestiva, the Sociedad Española de Endoscopia Digestiva and the Asociación Española de Ecografía Digestiva
Cookie policy Privacy Policy Legal Notice © Copyright 2023 y Creative Commons. The Spanish Journal of Gastroenterology