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

Afiliação

(1)    Instituto Alfa de Gastroenterologia, Hospital das Clínicas, Universidade Federal de Minas Gerais.

(2)    Hospital da Polícia Militar de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.

(3) Departamento de Gastroenterologia, Faculdade de Medicina da Universidade de São Paulo, São Paulo, São Paulo, Brazil.

(4) Disciplina de Gastroenterologia, Universidade Federal de São Paulo, São Paulo, São Paulo, Brazil.

(5) Hospital Universitário Clementino Fraga Filho e Departamento de Clínica Médica da Faculdade de Medicina, Universidade Federal Do Rio de Janeiro, Rio de Janeiro, Brazil.

(6) Instituto Alfa de Gastroenterologia, Hospital das Clínicas, Universidade Federal de Minas Gerais.

(7) Centro Universitário Lusíada-UNILUS, Santos, São Paulo, Brazil.

(8) Ambulatório Municipal de Hepatites Virais de São José Dos Campos, São José dos Campos, São Paulo, Brazil.

(9) Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, Bahia, Brazil.

(10) Divisão de Gastroenterologia (Gastrocentro), Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil.

(11) Hospital de Base do Distrito Federal, Brasília, Distrito Federal, Brazil.

(12) Serviço de Gastroenterologia, Hepatologia e Transplante Hepático, Hospital Nossa Senhora das Graças, Curitiba, Paraná, Brazil.

(13) Hospital Português, Salvador, Bahia, Brazil.

(14) Instituto de Gastroenterologia, Endoscopia e Proctologia, Uberlândia, Minas Gerais, Brazil.

(15) Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil.

(16) Serviço de Gastroenterologia e Hepatologia, Universidade Federal de Juiz de Fora, Juiz de Fora, Minas Gerais, Brazil.

(17) Hospital Universitário Cassiano Antônio Moraes, Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil.

(18) Irmandade da Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil.

(19) Escola Bahiana de Medicina e Saúde Pública, Salvador, Bahia, Brazil.

(20) Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, FL, USA.

Resumo

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.