Colangitis biliar primaria en EspañaResultados de un estudio Delphi sobre su epidemiología, diagnóstico, seguimiento y tratamiento
- Albert Parés 1
- Agustín Albillos Martínez 2
- Raúl J. Andrade Bellido 3
- Marina Berenguer Haym 4
- Javier Crespo García 5
- Manuel Romero-Gómez 6
- Mercedes Vergara Gómez 7
- Belén Vendrell 8
- Alicia Gil 9
- 1 Universidad de Barcelona. Barcelona
- 2 Universidad de Alcalá. Madrid
- 3 Complejo Hospitalario de Especialidades Virgen de la Victoria. Málaga
- 4 Hospital Universitari i Politècnic la Fe. Valencia
- 5 Hospital Universitario Marqués de Valdecilla. Santander
- 6 Hospital Virgen del Rocío. Sevilla
- 7 Universitat Autònoma de Barcelona. Sabadell, Barcelona
- 8 Intercept Pharmaceuticals. Madrid
- 9 Omakase Consulting. Barcelona
ISSN: 2340-416, 1130-0108
Ano de publicación: 2018
Volume: 110
Número: 10
Páxinas: 641-649
Tipo: Artigo
Outras publicacións en: Revista Española de Enfermedades Digestivas
Resumo
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.