Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population

Background and Aim The United Kingdom‐primary biliary cholangitis (UK‐PBC) and global primary biliary cholangitis group (GLOBE) prognostic models have been recently developed to predict long‐term outcomes in primary biliary cholangitis (PBC). However, these predictive scores have not yet been well e...

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Main Authors: Mohammad Alomari, Fahrettin Covut, Laith Al Momani, Pravallika Chadalavada, Asif Hitawala, Mark F Young, Carlos Romero‐Marrero
Format: Article
Language:English
Published: Wiley 2020-04-01
Series:JGH Open
Subjects:
Online Access:https://doi.org/10.1002/jgh3.12223
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author Mohammad Alomari
Fahrettin Covut
Laith Al Momani
Pravallika Chadalavada
Asif Hitawala
Mark F Young
Carlos Romero‐Marrero
author_facet Mohammad Alomari
Fahrettin Covut
Laith Al Momani
Pravallika Chadalavada
Asif Hitawala
Mark F Young
Carlos Romero‐Marrero
author_sort Mohammad Alomari
collection DOAJ
description Background and Aim The United Kingdom‐primary biliary cholangitis (UK‐PBC) and global primary biliary cholangitis group (GLOBE) prognostic models have been recently developed to predict long‐term outcomes in primary biliary cholangitis (PBC). However, these predictive scores have not yet been well evaluated in the U.S. population. Methods We retrospectively reviewed newly diagnosed PBC patients at the Cleveland Clinic between November 1998 and February 2017. Adverse events were defined as liver transplantation, liver‐related mortality, and all‐cause mortality. Transplant‐free survival (TFS) was estimated using the Kaplan–Meier method. Predictive performances of all prognostic models were evaluated using the C‐statistic. Results We identified 352 patients who used ursodeoxycholic acid therapy. Of them, 311 (88.4%) only had PBC, while 41 (11.6%) were diagnosed with PBC‐autoimmune hepatitis overlap. A total of 22 (6%), 47 (13%), and 55 (16%) patients had adverse events within 5, 10, and 15 years after diagnosis, respectively. In patients with PBC only, the C‐statistic in predicting 15‐year adverse events was 0.75 per GLOBE compared to 0.74 per UK‐PBC (P = 0.94), 0.73 per Rotterdam (P = 0.44), 0.66 per Barcelona (P = 0.004), 0.65 per Paris 1 (P = 0.005), 0.62 per Paris 2 (P < 0.0001), 0.60 per Toronto (P < 0.0001), and 0.60 per Mayo (P < 0.0001) scores. Median follow‐up was 9.2 years. Ten‐year TFS for patients who had optimal versus suboptimal treatment response was 92 versus 74% per Paris 1 (P < 0.0001), 95 versus 79% per Paris 2 (P = 0.0002), 93 versus 65% per Barcelona (P < 0.0001), and 96 versus 68% per Rotterdam (P < 0.0001) risk scores, respectively. Conclusion In our cohort of PBC patients, the UK‐PBC and GLOBE scores were both accurate and reasonably valid prognostic models in the U.S. population.
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spelling doaj.art-05f9830a13204450bffe137504f730dd2022-12-21T19:46:18ZengWileyJGH Open2397-90702020-04-014213213910.1002/jgh3.12223Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. populationMohammad Alomari0Fahrettin Covut1Laith Al Momani2Pravallika Chadalavada3Asif Hitawala4Mark F Young5Carlos Romero‐Marrero6Department of Internal Medicine Cleveland Clinic Foundation Cleveland Ohio USADepartment of Internal Medicine Cleveland Clinic Foundation Cleveland Ohio USADepartment of Internal Medicine East Tennessee State University Johnson City Tennessee USADepartment of Internal Medicine Cleveland Clinic Foundation Cleveland Ohio USADepartment of Internal Medicine Cleveland Clinic Foundation Cleveland Ohio USADepartment of Gastroenterology and Hepatology East Tennessee State University Johnson City Tennessee USADepartment of Gastroenterology Hepatology and Nutrition, Cleveland Clinic Foundation Cleveland Ohio USABackground and Aim The United Kingdom‐primary biliary cholangitis (UK‐PBC) and global primary biliary cholangitis group (GLOBE) prognostic models have been recently developed to predict long‐term outcomes in primary biliary cholangitis (PBC). However, these predictive scores have not yet been well evaluated in the U.S. population. Methods We retrospectively reviewed newly diagnosed PBC patients at the Cleveland Clinic between November 1998 and February 2017. Adverse events were defined as liver transplantation, liver‐related mortality, and all‐cause mortality. Transplant‐free survival (TFS) was estimated using the Kaplan–Meier method. Predictive performances of all prognostic models were evaluated using the C‐statistic. Results We identified 352 patients who used ursodeoxycholic acid therapy. Of them, 311 (88.4%) only had PBC, while 41 (11.6%) were diagnosed with PBC‐autoimmune hepatitis overlap. A total of 22 (6%), 47 (13%), and 55 (16%) patients had adverse events within 5, 10, and 15 years after diagnosis, respectively. In patients with PBC only, the C‐statistic in predicting 15‐year adverse events was 0.75 per GLOBE compared to 0.74 per UK‐PBC (P = 0.94), 0.73 per Rotterdam (P = 0.44), 0.66 per Barcelona (P = 0.004), 0.65 per Paris 1 (P = 0.005), 0.62 per Paris 2 (P < 0.0001), 0.60 per Toronto (P < 0.0001), and 0.60 per Mayo (P < 0.0001) scores. Median follow‐up was 9.2 years. Ten‐year TFS for patients who had optimal versus suboptimal treatment response was 92 versus 74% per Paris 1 (P < 0.0001), 95 versus 79% per Paris 2 (P = 0.0002), 93 versus 65% per Barcelona (P < 0.0001), and 96 versus 68% per Rotterdam (P < 0.0001) risk scores, respectively. Conclusion In our cohort of PBC patients, the UK‐PBC and GLOBE scores were both accurate and reasonably valid prognostic models in the U.S. population.https://doi.org/10.1002/jgh3.12223biliarycholangitiscirrhosisoverlap syndromeprognosisursodeoxycholic acid
spellingShingle Mohammad Alomari
Fahrettin Covut
Laith Al Momani
Pravallika Chadalavada
Asif Hitawala
Mark F Young
Carlos Romero‐Marrero
Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
JGH Open
biliary
cholangitis
cirrhosis
overlap syndrome
prognosis
ursodeoxycholic acid
title Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
title_full Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
title_fullStr Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
title_full_unstemmed Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
title_short Evaluation of the United Kingdom‐primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population
title_sort evaluation of the united kingdom primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the u s population
topic biliary
cholangitis
cirrhosis
overlap syndrome
prognosis
ursodeoxycholic acid
url https://doi.org/10.1002/jgh3.12223
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