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)
ERN RARE-LIVER and IAIHG Position paper, 2020: Second-line and third-line therapy for autoimmune hepatitis: https://www.sciencedirect.com/science/article/pii/S0168827820304700?via%3Dihub
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
(Including hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, Caroli disease, Caroli syndrome and peribiliary cysts)
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/
Sclerosing Cholangitis
EASL Guideline on Sclerosing Cholangitis, 2022: https://doi.org/10.1016/j.jhep.2022.05.011
(Including primary and secondary sclerosing cholangitis)
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
AASLD Practice Guidance, 2020: https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.31646
(Including Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease)
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/
—————————————————————————————————————————————————————————————————————————————————————
Liver Disease in Pregnancy
EASL Clinical Practice Guidelines on the management of liver diseases in pregnancy, 2023
https://easl.eu/publication-category/clinical-practice-guidelines/
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
EASL Quiz - Test Your Guidelines Knowledge
Test your Guidelines Knowledge – quick, handy, and helpful – to sharpen your skills. Each quiz presents a short case, drawing from EASL’s Clinical Practice Guidelines and relating to the latest in diagnostic and therapeutic developments. It poses a question and offers 4-5 possible answers to choose from. Pick your answer, check if it’s right, and compare with your peers’ responses.
>> Click here <<
ERN GUIDELINES Methodological Handbook
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