Revista espanola de enfermedades digestivas · 2018
Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment.
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Parés Albert, Albillos Agustín, Andrade Raul Jesús, Berenguer Marina, Crespo Javier, Romero-Gómez Manuel, Vergara Mercè, Vendrell Belén, Gil Alicia
Аннотация
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.
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.