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Multicenter Study
. 2021 Nov 10:2021:7746401.
doi: 10.1155/2021/7746401. eCollection 2021.

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population

Mateus Jorge Nardelli  1 , Paulo Lisboa Bittencourt  2   3 , Guilherme Grossi Lopes Cançado  1   4 , Luciana Costa Faria  1 , Cristiane Alves Villela-Nogueira  5 , Vivian Rotman  5 , Eliabe Silva de Abreu  1 , Fernanda Maria Farage Osório  1 , Andreia Silva Evangelista  5 , Liliana Sampaio Costa Mendes  6 , Daniel Ferraz de Campos Mazo  7 , Elodie Bonfim Hyppolito  8 , Adrielly de Souza Martins  9 , Liana Codes  2 , Izabelle Venturini Signorelli  10 , Geisa Perez Medina Gomide  11 , Luciana Agoglia  12 , Claudia Alexandra Pontes Ivantes  13 , Valéria Ferreira de Almeida E Borges  14 , Gabriela Perdomo Coral  15 , Rosamar Eulira Fontes Rezende  16 , Maria Lucia Gomes Ferraz  17 , Debora Raquel Benedita Terrabuio  18 , Eduardo Luiz Rachid Cançado  18 , Claudia Alves Couto  1
Affiliations
Multicenter Study

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population

Mateus Jorge Nardelli et al. Can J Gastroenterol Hepatol. .

Abstract

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.

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Conflict of interest statement

The authors declare that they no conflicts of interest.

Figures

Figure 1
Figure 1
Distribution of patients' age at PSC diagnosis according to sex (n = 177).
Figure 2
Figure 2
Kaplan–Meier curves of transplant-free survival of patients with PSC (n = 177).
See this image and copyright information in PMC

References

    1. Karlsen T. H., Folseraas T., Thorburn D., Vesterhus M. Primary sclerosing cholangitis – a comprehensive review. Journal of Hepatology . 2017;67(6):1298–1323. doi: 10.1016/j.jhep.2017.07.022. - DOI - PubMed
    1. Wiesner R. H., LaRusso N. F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology . 1980;79(2):200–206. doi: 10.1016/0016-5085(80)90131-6. - DOI - PubMed
    1. Kingham J. G. C., Kochar N., Gravenor M. B. Incidence, clinical patterns, and outcomes of primary sclerosing cholangitis in South Wales, United Kingdom. Gastroenterology . 2004;126(7):1929–1930. - PubMed
    1. Lindkvist B., Benito de Valle M., Gullberg B., Björnsson E. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology . 2010;52(2):571–577. doi: 10.1002/hep.23678. - DOI - PubMed
    1. Weismüller T. J., Trivedi P. J., Bergquist A., et al. Patient Age, sex, and inflammatory bowel disease phenotype Associate with course of primary sclerosing cholangitis. Gastroenterology . 2017;152(8):1975–e8. doi: 10.1053/j.gastro.2017.02.038. - DOI - PMC - PubMed

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