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. 2022 Jul;67(7):3305-3312.
doi: 10.1007/s10620-021-07122-y. Epub 2021 Jun 28.

Anti-mitochondrial Antibody-Negative Primary Biliary Cholangitis Is Part of the Same Spectrum of Classical Primary Biliary Cholangitis

Guilherme Grossi Lopes Cançado  1   2 , Michelle Harriz Braga  3 , Maria Lucia Gomes Ferraz  4 , Cristiane Alves Villela-Nogueira  5 , Debora Raquel Benedita Terrabuio  3 , Eduardo Luiz Rachid Cançado  3 , Mateus Jorge Nardelli  6 , Luciana Costa Faria  6 , Nathalia Mota de Faria Gomes  4 , Elze Maria Gomes Oliveira  7 , Vivian Rotman  5 , Maria Beatriz Oliveira  8 , Simone Muniz Carvalho Fernandes da Cunha  9 , Marlone Cunha-Silva  10 , Liliana Sampaio Costa Mendes  11 , Claudia Alexandra Pontes Ivantes  12 , Liana Codes  9   13 , Valéria Ferreira de Almeida E Borges  14   15 , Fabio Heleno de Lima Pace  16 , Mario Guimarães Pessoa  3 , Izabelle Venturini Signorelli  17 , Gabriela Perdomo Coral  18 , Paulo Lisboa Bittencourt  13   19 , Cynthia Levy  20 , Cláudia Alves Couto  6 ; Members of the Brazilian Cholestasis Study Group Consortium
Affiliations

Anti-mitochondrial Antibody-Negative Primary Biliary Cholangitis Is Part of the Same Spectrum of Classical Primary Biliary Cholangitis

Guilherme Grossi Lopes Cançado et al. Dig Dis Sci. 2022 Jul.

Abstract

Background: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease in which anti-mitochondrial antibodies (AMA) are the diagnostic hallmark. Whether AMA-negative PBC patients represent a different phenotype of disease is highly debated.

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.

Keywords: Anti-mitochondrial antibody; Autoantibody; Disease phenotype; Primary biliary cholangitis; Ursodeoxycholic acid.

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