OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT

Conflict of interest: No Introduction and Objectives: Primary biliary cholangitis (PBC) may present differently depending on various factors such as ethnicity and genetic background. Latin America has a highly admixed population with a unique genetic diversity compared to other regions of the world....

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Main Authors: Guilherme Grossi Lopes Cançado, Rafael Theodoro, Ezequiel Ridruejo, Lorena Castro Solari, Cristiane Alves Villela-Nogueira, Pablo Andres Coste Murillo, Harlim Rodríguez, Carlos Benítez Gajardo, Álvaro Urzúa, Eira Cerda Reyes, Paulo Lisboa Bittencourt, Alejandro Sosa, Emilia Vera, Luciana Costa Faria, Maria Lucia Ferraz, Mario Guimarães Pessoa, Debora Raquel Benedita Terrabuio, Eduardo Luiz Rachid Cançado, Claudia Alves Couto
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Annals of Hepatology
Online Access:http://www.sciencedirect.com/science/article/pii/S1665268124003855
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author Guilherme Grossi Lopes Cançado
Rafael Theodoro
Ezequiel Ridruejo
Lorena Castro Solari
Cristiane Alves Villela-Nogueira
Pablo Andres Coste Murillo
Harlim Rodríguez
Carlos Benítez Gajardo
Álvaro Urzúa
Eira Cerda Reyes
Paulo Lisboa Bittencourt
Alejandro Sosa
Emilia Vera
Luciana Costa Faria
Maria Lucia Ferraz
Mario Guimarães Pessoa
Debora Raquel Benedita Terrabuio
Eduardo Luiz Rachid Cançado
Claudia Alves Couto
author_facet Guilherme Grossi Lopes Cançado
Rafael Theodoro
Ezequiel Ridruejo
Lorena Castro Solari
Cristiane Alves Villela-Nogueira
Pablo Andres Coste Murillo
Harlim Rodríguez
Carlos Benítez Gajardo
Álvaro Urzúa
Eira Cerda Reyes
Paulo Lisboa Bittencourt
Alejandro Sosa
Emilia Vera
Luciana Costa Faria
Maria Lucia Ferraz
Mario Guimarães Pessoa
Debora Raquel Benedita Terrabuio
Eduardo Luiz Rachid Cançado
Claudia Alves Couto
author_sort Guilherme Grossi Lopes Cançado
collection DOAJ
description Conflict of interest: No Introduction and Objectives: Primary biliary cholangitis (PBC) may present differently depending on various factors such as ethnicity and genetic background. Latin America has a highly admixed population with a unique genetic diversity compared to other regions of the world. However, there is limited information available on the presentation and epidemiology of PBC in this region. This study aims to address the epidemiology of PBC in Latin America. Patients / Materials and Methods: Ongoing retrospective, international, multicentric cohort study sponsored by ALEH that enrolls PBC patients from different countries in Latin America. Results and Discussion: Data were accrued on 231 patients [Brazil (52%), Argentina (27.4%), Chile (10.8%), Costa Rica (4.5%), Cuba (3.6%), and Mexico (0.9%)], 92.1% female (mean age at diagnosis 50.5 years), 25.6% with cirrhosis at baseline. Overlap with autoimmune hepatitis was reported in 16.0% of cases. Most patients were symptomatic (67.9%) at diagnosis, with fatigue (41.9%) and pruritus (40.5%) being the main symptoms. Anti-mitochondrial antibodies (AMA) were positive in 70.8% and antinuclear antibodies (ANA) in 60.6%. Hashimoto thyroiditis (23.7%) and Sjogren syndrome (9.1%) were the most common extrahepatic autoimmune diseases associated with PBC. Mean baseline alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, and bilirubin levels were 445.9 (± 407), 89.8 (± 137.2), 37.6 (± 8.9) U/L, and 1.6 (± 3.1) mg/dL, respectively. Almost all patients (99.1%) were treated with ursodeoxycholic acid (UDCA). 67.4% achieved adequate response to UDCA according to the Toronto criteria and 32% normalized alkaline phosphatase at 12 months. Only 19.9% received second-line therapy, all with fibrates (89.1% bezafibrate, 8.7% ciprofibrate, 4.3% fenofibrate). Of the patients, 9% died, with 33% of deaths being liver-related, while 6% underwent liver transplantation. Hepatocellular carcinoma was diagnosed in 1.7% of patients. Conclusions: In this unprecedented study, the epidemiology of PBC in Latin America appears similar to that in other parts of the world. However, lower rates of AMA positivity were observed, and most patients were still diagnosed with symptomatic disease. Second-line therapy options were limited to the availability of fibrates only.
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spelling doaj-art-3711ee9973f54d9fa20a8d9408d6e7ec2024-12-07T08:25:20ZengElsevierAnnals of Hepatology1665-26812024-12-0129101602OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORTGuilherme Grossi Lopes Cançado0Rafael Theodoro1Ezequiel Ridruejo2Lorena Castro Solari3Cristiane Alves Villela-Nogueira4Pablo Andres Coste Murillo5Harlim Rodríguez6Carlos Benítez Gajardo7Álvaro Urzúa8Eira Cerda Reyes9Paulo Lisboa Bittencourt10Alejandro Sosa11Emilia Vera12Luciana Costa Faria13Maria Lucia Ferraz14Mario Guimarães Pessoa15Debora Raquel Benedita Terrabuio16Eduardo Luiz Rachid Cançado17Claudia Alves Couto18Universidade Federal de Minas Gerais, Belo Horizonte, BrasilUniversidade Federal de Minas Gerais, Belo Horizonte, BrasilCentro de Educación Médica e Investigaciones Clínicas Norberto Quirno, Buenos Aires, ArgentinaClinica Universidad de Los Andes, Santiago, ChileUniversidade Federal do Rio de Janeiro, Rio de Janeiro, BrasilHospital R. A. Calderón Guardia, San José, Costa RicaInstituto de Gastroenterología de Cuba, Habana, CubaUC Christus, Santiago, ChileUniversidad de Chile, Santiago, ChileHospital Militar Central México, Ciudad de México, MéxicoHospital Portugues, Salvador, BrasilPontificia Universidad Católica de Chile, Santiago, ChileHospital Regional Guayaquil, Guayaquil, EcuadorUniversidade Federal de Minas Gerais, Belo Horizonte, BrasilUniversidade Federal de São Paulo, São Paulo, BrasilUniversidade de São Paulo, São Paulo, BrasilUniversidade de São Paulo, São Paulo, BrasilUniversidade de São Paulo, São Paulo, BrasilUniversidade Federal de Minas Gerais, Belo Horizonte, BrasilConflict of interest: No Introduction and Objectives: Primary biliary cholangitis (PBC) may present differently depending on various factors such as ethnicity and genetic background. Latin America has a highly admixed population with a unique genetic diversity compared to other regions of the world. However, there is limited information available on the presentation and epidemiology of PBC in this region. This study aims to address the epidemiology of PBC in Latin America. Patients / Materials and Methods: Ongoing retrospective, international, multicentric cohort study sponsored by ALEH that enrolls PBC patients from different countries in Latin America. Results and Discussion: Data were accrued on 231 patients [Brazil (52%), Argentina (27.4%), Chile (10.8%), Costa Rica (4.5%), Cuba (3.6%), and Mexico (0.9%)], 92.1% female (mean age at diagnosis 50.5 years), 25.6% with cirrhosis at baseline. Overlap with autoimmune hepatitis was reported in 16.0% of cases. Most patients were symptomatic (67.9%) at diagnosis, with fatigue (41.9%) and pruritus (40.5%) being the main symptoms. Anti-mitochondrial antibodies (AMA) were positive in 70.8% and antinuclear antibodies (ANA) in 60.6%. Hashimoto thyroiditis (23.7%) and Sjogren syndrome (9.1%) were the most common extrahepatic autoimmune diseases associated with PBC. Mean baseline alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, and bilirubin levels were 445.9 (± 407), 89.8 (± 137.2), 37.6 (± 8.9) U/L, and 1.6 (± 3.1) mg/dL, respectively. Almost all patients (99.1%) were treated with ursodeoxycholic acid (UDCA). 67.4% achieved adequate response to UDCA according to the Toronto criteria and 32% normalized alkaline phosphatase at 12 months. Only 19.9% received second-line therapy, all with fibrates (89.1% bezafibrate, 8.7% ciprofibrate, 4.3% fenofibrate). Of the patients, 9% died, with 33% of deaths being liver-related, while 6% underwent liver transplantation. Hepatocellular carcinoma was diagnosed in 1.7% of patients. Conclusions: In this unprecedented study, the epidemiology of PBC in Latin America appears similar to that in other parts of the world. However, lower rates of AMA positivity were observed, and most patients were still diagnosed with symptomatic disease. Second-line therapy options were limited to the availability of fibrates only.http://www.sciencedirect.com/science/article/pii/S1665268124003855
spellingShingle Guilherme Grossi Lopes Cançado
Rafael Theodoro
Ezequiel Ridruejo
Lorena Castro Solari
Cristiane Alves Villela-Nogueira
Pablo Andres Coste Murillo
Harlim Rodríguez
Carlos Benítez Gajardo
Álvaro Urzúa
Eira Cerda Reyes
Paulo Lisboa Bittencourt
Alejandro Sosa
Emilia Vera
Luciana Costa Faria
Maria Lucia Ferraz
Mario Guimarães Pessoa
Debora Raquel Benedita Terrabuio
Eduardo Luiz Rachid Cançado
Claudia Alves Couto
OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
Annals of Hepatology
title OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
title_full OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
title_fullStr OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
title_full_unstemmed OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
title_short OP- 4 EPIDEMIOLOGY OF PRIMARY BILIARY CHOLANGITIS IN LATIN AMERICA: PRELIMINARY RESULTS FROM ALLATIN COHORT
title_sort op 4 epidemiology of primary biliary cholangitis in latin america preliminary results from allatin cohort
url http://www.sciencedirect.com/science/article/pii/S1665268124003855
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