Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment

Albert Parés, Agustín Albillos, Raúl J. Andrade, Marina Berenguer, Javier Crespo, Manuel Romero-Gómez, Mercè Vergara, Belén Vendrell, Alicia Gil

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© 2018. SEPD. Introduction: primary biliary cholangitis (PBC) is a rare disease with limited data regarding its epidemiology and standard clinical management in Spain. Objective: to gain insight into the epidemiology, patient flow, diagnosis, follow-up and treatment of PBC in Spain. Methods: a review of the literature and Delphi study involving 28 specialists in two rounds of consultations and an in-person results validation workshop. Results: there are approximately 9,400 patients with PBC in Spain, with an annual incidence of 0.51-3.86 cases/100,000 population. Albeit, a high error margin may be presumed due to the scarcity of relevant studies on this subject. Several months may elapse from suspicion to a confirmed diagnosis, usually by a gastroenterologist or hepatologist. The role of the liver biopsy for diagnosis and follow-up is heterogeneous. Overall, 95% of patients are treated with ursodeoxycholic acid (UDCA) and response is primarily monitored using the Barcelona criteria. Follow-up is performed every six months, with a heterogeneous use of the various available techniques. No recommendations or second-line commercial drugs are available in the case of no response, inadequate response or intolerance to UDCA. Conclusions: while epidemiology may be estimated based on expert opinions, national registries are needed to provide accurate, up-to-date information on epidemiological parameters, disease stage and response to treatment in patients with PBC. Furthermore, novel therapies are required for selected patient groups.
Original languageEnglish
Pages (from-to)641-649
JournalRevista Espanola de Enfermedades Digestivas
Issue number10
Publication statusPublished - 1 Jan 2018


  • Diagnosis
  • Epidemiology
  • Primary biliary cholangitis
  • Treatment


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