Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP

Cholestatic liver diseases comprise a heterogeneous group of disorders affecting both adult and pediatric population, characterized by alterations in bile formation, secretion, or flow, leading to the accumulation of bile acids and other toxic substances in the liver. In recent years, advances in new pharmacological therapies, the availability of next-generation genetic sequencing techniques, and the development of specific treatments for genetic cholestasis have transformed the diagnostic and therapeutic approach to these conditions. This document, jointly prepared by the "Asociación Española para el Estudio del Hígado" (AEEH) and the "Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica" (SEGHNP), presents a national evidence-based guideline for the diagnosis and management of hepatic cholestasis in Spain. It addresses recommendations for differential diagnosis, diagnostic algorithms, indications for genetic studies, treatment and follow-up criteria in diseases such as primary biliary cholangitis, primary sclerosing cholangitis, genetic cholestasis, intrahepatic cholestasis of pregnancy, and vanishing bile duct syndrome. In addition, recommendations are included for the management of extrahepatic complications, indications for liver transplantation, and special considerations in pregnancy and childhood. The guideline emphasizes the importance of a multidisciplinary approach, the use of non-invasive tools for risk stratification, and the incorporation of new targeted therapies, with the aim of improving the prognosis and quality of life of patients affected by cholestatic liver diseases.

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Publication Details

Journal
Gastroenterología y Hepatología
Published
2025-11-01
DOI
https://doi.org/10.1016/j.gastrohep.2025.502629
Primary Topic
Liver Diseases and Immunity
Type
article
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article

Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP

Antonio Olveira, Gema Muñoz‐Bartolo, Álvaro Díaz‐González, Jesús Quintero et al.
Gastroenterología y Hepatología
Liver Diseases and Immunity
article

Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP

Antonio Olveira, Gema Muñoz‐Bartolo, Álvaro Díaz‐González, Jesús Quintero, Francisco Jorquera, Sergio Rodríguez‐Tajes, Conrado Fernández‐Rodríguez, Javier Ampuero, María Alós Díez, Cristina Molera, Marina Berenguer, J.L. Montero, María‐Carlota Londoño, Rosa Maria Morillas, Elena Gómez Domínguez, Esther Molina, Marta Casado, Luisa García Buey
article en

Abstract

Cholestatic liver diseases comprise a heterogeneous group of disorders affecting both adult and pediatric population, characterized by alterations in bile formation, secretion, or flow, leading to the accumulation of bile acids and other toxic substances in the liver. In recent years, advances in new pharmacological therapies, the availability of next-generation genetic sequencing techniques, and the development of specific treatments for genetic cholestasis have transformed the diagnostic and therapeutic approach to these conditions. This document, jointly prepared by the "Asociación Española para el Estudio del Hígado" (AEEH) and the "Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica" (SEGHNP), presents a national evidence-based guideline for the diagnosis and management of hepatic cholestasis in Spain. It addresses recommendations for differential diagnosis, diagnostic algorithms, indications for genetic studies, treatment and follow-up criteria in diseases such as primary biliary cholangitis, primary sclerosing cholangitis, genetic cholestasis, intrahepatic cholestasis of pregnancy, and vanishing bile duct syndrome. In addition, recommendations are included for the management of extrahepatic complications, indications for liver transplantation, and special considerations in pregnancy and childhood. The guideline emphasizes the importance of a multidisciplinary approach, the use of non-invasive tools for risk stratification, and the incorporation of new targeted therapies, with the aim of improving the prognosis and quality of life of patients affected by cholestatic liver diseases.

Gastroenterología y Hepatología
Hospital Sant Joan de Déu Barcelona (ES), Hospital Universitario La Paz (ES), Hospital General Universitario Gregorio Marañón (ES), Complejo Hospitalario Universitario de Santiago (ES), Marqués de Valdecilla University Hospital (ES), Hospital Universitario Fundación Alcorcón (ES), Hospital Universitario Rey Juan Carlos (ES), Hospital Universitario 12 De Octubre (ES), Instituto de Investigación Marqués de Valdecilla (ES), Hospital Universitario Reina Sofía (ES), Vall d'Hebron Hospital Universitari (ES), Hospital Universitario de La Princesa (ES), Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (ES), Complejo Hospitalario Torrecárdenas (ES), Hospital Universitario Virgen del Rocío (ES), Fundació Clínic per a la Recerca Biomèdica (ES), Complejo Asistencial Universitario de Palencia (ES), Universidad Autónoma de Madrid (ES), Universitat de Barcelona (ES)
Openalex Percentile: Top 55%
Liver Diseases and Immunity
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