Clinical features and treatment outcomes of primary biliary cholangitis in a highly admixed population.
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Cançado Guilherme Grossi Lopes, Braga Michelle Harriz, Ferraz Maria Lúcia Gomes, Villela-Nogueira Cristiane Alves, Terrabuio Debora Raquel Benedita, Cançado Eduardo Luiz Rachid, Nardelli Mateus Jorge, Faria Luciana Costa, Gomes Nathalia Mota de Faria, de Oliveira Elze Maria Gomes, Rotman Vivian, de Oliveira Maria Beatriz, da Cunha Simone Muniz Carvalho Fernandes, Mazo Daniel Ferraz de Campos, Mendes Liliana Sampaio Costa, Ivantes Claudia Alexandra Pontes, Codes Liana, de Almeida E Borges Valéria Ferreira, Pace Fabio Heleno de Lima, Pessoa Mario Guimarães, Signorelli Izabelle Venturini, Coral Gabriela Perdomo, Bittencourt Paulo Lisboa, Levy Cynthia, Couto Cláudia Alves, Members of the Brazilian Cholestasis Study Group Consortium
Аннотация
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.