Clinical practice guidelines and position papers

One of the tasks set by the European Commission for the ERNs is to contribute towards advancing clinical care in rare liver diseases by developing practice guidelines and position papers. We are pleased to collaborate with EASL and ESPGHAN in this regard to ensure that our activities are mutually beneficial and that we share knowledge and experience in the most effective way.

Please find below a list of guidelines and position papers endorsed by ERN RARE-LIVER:

Acute Liver Failure

EASL Guideline, 2017: https://easl.eu/publication/acute-liver-failure-guidelines/

Alpha-1 antitrypsin deficiency (AATD)

EASL Review, 2022: https://www.journal-of-hepatology.eu/article/S0168-8278(21)02227-3/fulltext

Autoimmune Hepatitis (AIH)

ESPGHAN Guideline for paediatric patients, 2019: https://espghan.info/files/Diagnosis-and-Management-of-Paediatric-Autoimmune-Hepatitis-AIH.-ESPGHAN-Advice-Guide.-2019.-Ver1..pdf

EASL Guideline, 2015: https://easl.eu/publication/autoimmune-hepatitis/

Cystic Liver Diseases

EASL Guideline, 2022: https://doi.org/10.1016/j.jhep.2022.06.002

Genetic Cholestatic Diseases

NASPGHAN and ESPGHAN Guideline on Cholestatic Jaundice in Infants, 2017: https://www.naspghan.org/files/documents/pdfs/position-papers/Guideline_for_the_Evaluation_of_Cholestatic.23.pdf

EASL Guideline on cholestatic-liver-diseases, 2009: https://www.sciencedirect.com/science/article/pii/S0168827809003092?via%3Dihub

Haemochromatosis

EASL Guideline, 2022: https://www.journal-of-hepatology.eu/article/S0168-8278(22)00211-2/fulltext#secsectitle0115

Intrahepatic Cholangiocarcinoma

Consensus Statement on CCA, 2020: https://doi.org/10.1038/s41575-020-0310-z

EASL Guideline, 2014: https://www.journal-of-hepatology.eu/article/S0168-8278(14)00067-1/pdf

Intrahepatic cholestasis of pregnancy; HELLP syndrome

EASL Guideline on cholestatic-liver-diseases, 2009: https://www.sciencedirect.com/science/article/pii/S0168827809003092?via%3Dihub

Primary Biliary Cholangitis (PBC)

EASL Guideline, 2017: https://easl.eu/publication/the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis/

Primary Sclerosing Cholangitis (PSC)

EASL Guideline on Sclerosing Cholangitis, 2022: https://doi.org/10.1016/j.jhep.2022.05.011

ESGE and EASL Guideline, 2017: https://doi.org/10.1055/s-0043-107029

EASL Guideline on cholestatic-liver-diseases, 2009: https://www.sciencedirect.com/science/article/pii/S0168827809003092?via%3Dihub

Vascular Liver Diseases

AFEF and ERN RARE-LIVER Position papers, 2020: https://doi.org/10.1016/j.clinre.2020.03.009

EASL Guideline, 2015: https://easl.eu/publication/vascular-diseases-of-the-liver/

Wilson Disease

ESPGHAN Guideline, 2018: http://www.espghan.org/fileadmin/user_upload/guidelines_pdf/Hep_Nutr/Wilson_s_Disease_in_Children___A_Position_Paper_by.32.pdf

EASL Guideline, 2012: https://easl.eu/publication/management-of-wilsons-disease/

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Liver Transplantation

ESPGHAN Guideline on LTX for paediatric Autoimmune Liver Diseases, 2019: https://www.espghan.org/dam/jcr:77c33feb-7b11-4540-af8c-775622bdcb9f/2019_Liver_Transplantation__LT__for_Paediatric_Autoimmune_Liver_Disease._ESPGHAN_Advice_Guide.pdf

EASL Guideline, 2015: https://easl.eu/publication/liver-transplantation/

Transition from paediatric to adult care

ESPGHAN Guideline, 2018: http://www.espghan.org/fileadmin/user_upload/Society_Papers/The_Health_Care_Transition_of_Youth_With_Liver.29.pdf

The presented methodology is based on the current international “gold standards” that have been developed along the years and used by key international and national bodies and organisations specialised in the development of Clinical Practice Guidelines and other Clinical Decision Support Tools.

This methodology will ensure the quality and standardisation of the CPG and CDST produced and used by the ERNs, the backbone of the process of the diagnosis and treatment of patients suffering of rare or low prevalence and complex diseases.

The handbook includes 12 separated guidelines addressing the following conceptual and methodological aspects of the programme.

0# Introduction to the handbook
1# Prioritisation
2# Appraisal
3# Adaptation & Adoption
4# Elaboration of CPG
5# Elaboration of Clinical Consensus Statements
6# Elaboration of Evidence Reports
7# Elaboration of Pathways
8# Elaboration of Evidence Based Protocols
9# Elaboration of Do’s & Don’ts Factsheets
10# Elaboration of Quality Measures
11# Elaboration of Patient Information Booklets
12# Evaluation of Uptake of CPGs and CDST