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Treatment response in primary biliary cholangitis: The role of autoimmune hepatitis features.
Mané, Fernando; Cardoso, Hélder; Liberal, Rodrigo; Lopes, Susana; Pereira, Pedro; Gonçalves, Regina; Macedo, Guilherme.
Afiliação
  • Mané F; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal.
  • Cardoso H; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal. Electronic address: hcmail@sapo.pt.
  • Liberal R; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal.
  • Lopes S; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal.
  • Pereira P; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal.
  • Gonçalves R; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal.
  • Macedo G; Gastroenterology Department, Faculty of Medicine, University of Porto, Porto, Portugal; Gastroenterology Department, Hospitalar Center of São João, Porto, Portugal.
Ann Hepatol ; 18(3): 488-493, 2019.
Article em En | MEDLINE | ID: mdl-31036497
INTRODUCTION AND AIM: Primary biliary cholangitis is a rare disease with scarce epidemiological data in Southern Europe. The authors aimed to evaluate treatment response in a cohort of patients. MATERIALS AND METHODS: This retrospective observational single-centre study included patients with diagnostic criteria of primary biliary cholangitis. Data on disease presentation, laboratory results, treatment and clinical endpoints were collected and analyzed. RESULTS: Fifty-three patients were included, 89% women, with mean age of 62±15 years at diagnosis. The majority was asymptomatic (49%), tested positive for antimitochondrial antibodies (96%) and had increased alkaline phosphatase (median=214U/L). 75% of the patients had liver histology and the majority were in Ludwig's stage I (42%). Autoimmune hepatitis (AIH) features were found in seven patients (13%). All were treated with ursodeoxycholic acid (UDCA) and 56% achieved biochemical response at one year; patients with AIH features exhibited steeper decreases in alkaline phosphatase (p=0.007) and reached the endpoint of 40% decrease in alkaline phosphatase more frequently (p=0.017). CONCLUSION: In conclusion a significant proportion of patients failed to achieve an adequate response to UDCA treatment. The response rate of patients with AIH features was better, which could be related to a different phenotype or to the potential impact of immunosuppressive agents.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Ácido Ursodesoxicólico / Hepatite Autoimune / Imunossupressores / Fígado / Cirrose Hepática Biliar Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Hepatol Assunto da revista: GASTROENTEROLOGIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Portugal País de publicação: México

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Ácido Ursodesoxicólico / Hepatite Autoimune / Imunossupressores / Fígado / Cirrose Hepática Biliar Tipo de estudo: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Ann Hepatol Assunto da revista: GASTROENTEROLOGIA Ano de publicação: 2019 Tipo de documento: Article País de afiliação: Portugal País de publicação: México