Clinical features and treatment outcomes of primary biliary cholangitis in a highly admixed population.

Autor:	Cançado GGL; Instituto Alfa de Gastroenterologia, Hospital das Clínicas da Universidade Federal de Minas Gerais, Av. Professor Alfredo Balena 110, Belo Horizonte 30130-100, Minas Gerais, Brazil; Hospital da Polícia Militar de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil. Electronic address: guilhermegrossi@terra.com.br., Braga MH; Departamento de Gastroenterologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Ferraz MLG; Disciplina de Gastroenterologia, Universidade Federal de São Paulo, São Paulo, Brazil., Villela-Nogueira CA; Hospital Universitário Clementino Fraga Filho e Departamento de Clínica Médica da Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil., Terrabuio DRB; Departamento de Gastroenterologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Cançado ELR; Departamento de Gastroenterologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Nardelli MJ; Instituto Alfa de Gastroenterologia, Hospital das Clínicas da Universidade Federal de Minas Gerais, Av. Professor Alfredo Balena 110, Belo Horizonte 30130-100, Minas Gerais, Brazil., Faria LC; Instituto Alfa de Gastroenterologia, Hospital das Clínicas da Universidade Federal de Minas Gerais, Av. Professor Alfredo Balena 110, Belo Horizonte 30130-100, Minas Gerais, Brazil., Gomes NMF; Disciplina de Gastroenterologia, Universidade Federal de São Paulo, São Paulo, Brazil., de Oliveira EMG; Centro Universitário Lusíada - UNILUS, Santos, São Paulo, Brazil., Rotman V; Hospital Universitário Clementino Fraga Filho e Departamento de Clínica Médica da Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil., de Oliveira MB; Ambulatório Municipal de Hepatites Virais de São José dos Campos, São José dos Campos, São Paulo, Brazil., da Cunha SMCF; Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, Bahia, Brazil., Mazo DFC; Divisão de Gastroenterologia (Gastrocentro), Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil., Mendes LSC; Hospital de Base do Distrito Federal, Brasília, Distrito Federal, Brazil., Ivantes CAP; Serviço de Gastroenterologia, Hepatologia e Transplante Hepático, Hospital Nossa Senhora das Graças, Curitiba, Paraná, Brazil., Codes L; Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, Bahia, Brazil; Hospital Português, Salvador, Bahia, Brazil., de Almeida E Borges VF; Instituto de Gastroenterologia, Endoscopia e Proctologia, Uberlândia, Minas Gerais, Brazil; Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil., Pace FHL; Serviço de Gastroenterologia e Hepatologia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brazil., Pessoa MG; Departamento de Gastroenterologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Signorelli IV; Hospital Universitário Cassiano Antônio Moraes, Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil., Coral GP; Irmandade da Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil., Bittencourt PL; Hospital Português, Salvador, Bahia, Brazil; Escola Bahiana de Medicina e Saúde Pública, Salvador, Bahia, Brazil., Levy C; Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, United States., Couto CA; Instituto Alfa de Gastroenterologia, Hospital das Clínicas da Universidade Federal de Minas Gerais, Av. Professor Alfredo Balena 110, Belo Horizonte 30130-100, Minas Gerais, Brazil.
Jazyk:	angličtina
Zdroj:	Annals of hepatology [Ann Hepatol] 2022 Jan-Feb; Vol. 27 (1), pp. 100546. Date of Electronic Publication: 2021 Sep 30.
DOI:	10.1016/j.aohep.2021.100546
Abstrakt:	Introduction and Objectives: Little is known about primary biliary cholangitis (PBC) in non-whites. The purpose of this study was to evaluate clinical features and outcomes of PBC in a highly admixed population. Material and Methods: The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical features and treatment outcomes of Brazilian patients with PBC. Results: 562 patients (95% females, mean age 51 ± 11 years) with PBC were included. Concurrent autoimmune diseases and overlap with autoimmune hepatitis (AIH) occurred, respectively, in 18.9% and 14%. After a mean follow-up was 6.2 ± 5.3 years, 32% had cirrhosis, 7% underwent liver transplantation and 3% died of liver-related causes. 96% were treated with ursodeoxycholic acid (UDCA) and 12% required add-on therapy with fibrates, either bezafibrate, fenofibrate or ciprofibrate. Response to UDCA and to UDCA/fibrates therapy varied from 39%-67% and 42-61%, respectively, according to different validated criteria. Advanced histological stages and non-adherence to treatment were associated with primary non-response to UDCA, while lower baseline alkaline phosphatase (ALP) and aspartate aminotransferase (AST) levels correlated with better responses to both UDCA and UDCA/fibrates. Conclusions: Clinical features of PBC in highly admixed Brazilians were similar to those reported in Caucasians and Asians, but with inferior rates of overlap syndrome with AIH. Response to UDCA was lower than expected and inversely associated with histological stage and baseline AST and ALP levels. Most of patients benefited from add-on fibrates, including ciprofibrate. A huge heterogeneity in response to UDCA therapy according to available international criteria was observed and reinforces the need of global standardization. Competing Interests: Conflicts of interest None. (Copyright © 2021 Fundación Clínica Médica Sur, A.C. Published by Elsevier España, S.L.U. All rights reserved.)
Databáze:	MEDLINE
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