Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population
Background. Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods....
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2021
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oai:doaj.org-article:66ca1b1c7ff64ccca760bc031a2271722021-11-22T01:11:04ZClinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population2291-279710.1155/2021/7746401https://doaj.org/article/66ca1b1c7ff64ccca760bc031a2271722021-01-01T00:00:00Zhttp://dx.doi.org/10.1155/2021/7746401https://doaj.org/toc/2291-2797Background. Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods. Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results. This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21–42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion. Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline.Mateus Jorge NardelliPaulo Lisboa BittencourtGuilherme Grossi Lopes CançadoLuciana Costa FariaCristiane Alves Villela-NogueiraVivian RotmanEliabe Silva de AbreuFernanda Maria Farage OsórioAndreia Silva EvangelistaLiliana Sampaio Costa MendesDaniel Ferraz de Campos MazoElodie Bonfim HyppolitoAdrielly de Souza MartinsLiana CodesIzabelle Venturini SignorelliGeisa Perez Medina GomideLuciana AgogliaClaudia Alexandra Pontes IvantesValéria Ferreira de Almeida e BorgesGabriela Perdomo CoralRosamar Eulira Fontes RezendeMaria Lucia Gomes FerrazDebora Raquel Benedita TerrabuioEduardo Luiz Rachid CançadoClaudia Alves CoutoHindawi LimitedarticleDiseases of the digestive system. GastroenterologyRC799-869ENCanadian Journal of Gastroenterology and Hepatology, Vol 2021 (2021) |
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DOAJ |
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DOAJ |
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EN |
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Diseases of the digestive system. Gastroenterology RC799-869 |
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Diseases of the digestive system. Gastroenterology RC799-869 Mateus Jorge Nardelli Paulo Lisboa Bittencourt Guilherme Grossi Lopes Cançado Luciana Costa Faria Cristiane Alves Villela-Nogueira Vivian Rotman Eliabe Silva de Abreu Fernanda Maria Farage Osório Andreia Silva Evangelista Liliana Sampaio Costa Mendes Daniel Ferraz de Campos Mazo Elodie Bonfim Hyppolito Adrielly de Souza Martins Liana Codes Izabelle Venturini Signorelli Geisa Perez Medina Gomide Luciana Agoglia Claudia Alexandra Pontes Ivantes Valéria Ferreira de Almeida e Borges Gabriela Perdomo Coral Rosamar Eulira Fontes Rezende Maria Lucia Gomes Ferraz Debora Raquel Benedita Terrabuio Eduardo Luiz Rachid Cançado Claudia Alves Couto Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| description |
Background. Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods. Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results. This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21–42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion. Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline. |
| format |
article |
| author |
Mateus Jorge Nardelli Paulo Lisboa Bittencourt Guilherme Grossi Lopes Cançado Luciana Costa Faria Cristiane Alves Villela-Nogueira Vivian Rotman Eliabe Silva de Abreu Fernanda Maria Farage Osório Andreia Silva Evangelista Liliana Sampaio Costa Mendes Daniel Ferraz de Campos Mazo Elodie Bonfim Hyppolito Adrielly de Souza Martins Liana Codes Izabelle Venturini Signorelli Geisa Perez Medina Gomide Luciana Agoglia Claudia Alexandra Pontes Ivantes Valéria Ferreira de Almeida e Borges Gabriela Perdomo Coral Rosamar Eulira Fontes Rezende Maria Lucia Gomes Ferraz Debora Raquel Benedita Terrabuio Eduardo Luiz Rachid Cançado Claudia Alves Couto |
| author_facet |
Mateus Jorge Nardelli Paulo Lisboa Bittencourt Guilherme Grossi Lopes Cançado Luciana Costa Faria Cristiane Alves Villela-Nogueira Vivian Rotman Eliabe Silva de Abreu Fernanda Maria Farage Osório Andreia Silva Evangelista Liliana Sampaio Costa Mendes Daniel Ferraz de Campos Mazo Elodie Bonfim Hyppolito Adrielly de Souza Martins Liana Codes Izabelle Venturini Signorelli Geisa Perez Medina Gomide Luciana Agoglia Claudia Alexandra Pontes Ivantes Valéria Ferreira de Almeida e Borges Gabriela Perdomo Coral Rosamar Eulira Fontes Rezende Maria Lucia Gomes Ferraz Debora Raquel Benedita Terrabuio Eduardo Luiz Rachid Cançado Claudia Alves Couto |
| author_sort |
Mateus Jorge Nardelli |
| title |
Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| title_short |
Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| title_full |
Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| title_fullStr |
Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| title_full_unstemmed |
Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population |
| title_sort |
clinical features and outcomes of primary sclerosing cholangitis in the highly admixed brazilian population |
| publisher |
Hindawi Limited |
| publishDate |
2021 |
| url |
https://doaj.org/article/66ca1b1c7ff64ccca760bc031a227172 |
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