Anti-mitochondrial Antibody-Negative Primary Biliary Cholangitis Is Part of the Same Spectrum of Classical Primary Biliary Cholangitis.
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Cançado Guilherme Grossi Lopes, Braga Michelle Harriz, Ferraz Maria Lucia Gomes, Villela-Nogueira Cristiane Alves, Terrabuio Debora Raquel Benedita, Cançado Eduardo Luiz Rachid, Nardelli Mateus Jorge, Faria Luciana Costa, de Faria Gomes Nathalia Mota, Oliveira Elze Maria Gomes, Rotman Vivian, Oliveira Maria Beatriz, da Cunha Simone Muniz Carvalho Fernandes, Cunha-Silva Marlone, Mendes Liliana Sampaio Costa, Ivantes Claudia Alexandra Pontes, Codes Liana, de Almeida E Borges Valéria Ferreira, de Lima Pace Fabio Heleno, 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
AIMS: The purpose of our study was to compare AMA-positive and AMA-negative PBC patients in a large non-white admixed Brazilian cohort.
METHODS: The Brazilian Cholestasis Study Group multicentre database was reviewed to assess demographics, clinical features and treatment outcomes of Brazilian PBC patients, stratifying data according to AMA status.
RESULTS: A total of 464 subjects (95.4% females, mean age 56 ± 5 years) with PBC were included. Three hundred and eighty-four (83%) subjects were AMA-positive, whereas 80 (17%) had AMA-negative PBC. Subjects with AMA-negative PBC were significantly younger (52.2 ± 14 vs. 59.6 ± 11 years, p = 0.001) and had their first symptom at an earlier age (43.2 ± 13 vs. 49.5 ± 12 years, p = 0.005). Frequency of type 2 diabetes was significantly increased in subjects with AMA-negative PBC (22.5% vs. 12.2%, p = 0.03). Lower IgM (272.2 ± 183 vs. 383.2 ± 378 mg/dL, p = 0.01) and triglycerides (107.6 ± 59.8 vs.129.3 ± 75.7 mg/dL, p = 0.025) and higher bilirubin (3.8 ± 13.5 vs. 1.8 ± 3.4 mg/dL, p = 0.02) levels were also observed in this subgroup. Response to ursodeoxycholic acid varied from 40.5 to 63.3% in AMA-positive and 34 to 62.3% in AMA-negative individuals, according to different response criteria. Outcomes such as development of liver-related complications, death and requirement for liver transplantation were similar in both groups.
CONCLUSIONS: AMA-negative PBC patients are similar to their AMA-positive counterparts with subtle differences observed in clinical and laboratory features.