Article
Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment
Author/s | Parés, Albert
Albillos, Agustín Andrade, Raúl Jesús Berenguer, Marina Crespo, Javier Romero Gómez, Manuel Vergara, Mercè Vendrell, Belén Gil, Alicia |
Department | Universidad de Sevilla. Departamento de Medicina |
Publication Date | 2018 |
Deposit Date | 2024-05-16 |
Published in |
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Abstract | 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
f
... 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 f low, diagnosis, follow-up and treatment of PBC in Spain. Methods: a review of the literature and Delphi study involv ing 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. Sev eral 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 per formed 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 pro vide accurate, up-to-date information on epidemiologi cal parameters, disease stage and response to treatment in patients with PBC. Furthermore, novel therapies are required for selected patient groups. |
Citation | Parés, A., Albillos, A., Andrade, R.J., Berenguer, M., Crespo, J., Romero Gómez, M.,...,Gil, A. (2018). Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment. Revista Española de Enfermedades Digestivas, 110 (10), 641-649. https://doi.org/10.17235/reed.2018.5665/2018. |
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