TY - JOUR
T1 - Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment
AU - Parés, Albert
AU - Albillos, Agustín
AU - Andrade, Raúl J.
AU - Berenguer, Marina
AU - Crespo, Javier
AU - Romero-Gómez, Manuel
AU - Vergara, Mercè
AU - Vendrell, Belén
AU - Gil, Alicia
PY - 2018/1/1
Y1 - 2018/1/1
N2 - © 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.
AB - © 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.
KW - Diagnosis
KW - Epidemiology
KW - Primary biliary cholangitis
KW - Treatment
U2 - 10.17235/reed.2018.5665/2018
DO - 10.17235/reed.2018.5665/2018
M3 - Article
C2 - 30032637
VL - 110
SP - 641
EP - 649
JO - Revista Espanola de Enfermedades Digestivas
JF - Revista Espanola de Enfermedades Digestivas
SN - 1130-0108
IS - 10
ER -