Single Topic Conference on Autoimmune Liver Disease from the Canadian Association for the Study of the Liver.
autoimmune hepatitis
cirrhosis
overlap syndrome
primary biliary cirrhosis
primary sclerosing cholangitis.
Journal
Canadian liver journal
ISSN: 2561-4444
Titre abrégé: Can Liver J
Pays: Canada
ID NLM: 101778326
Informations de publication
Date de publication:
2021
2021
Historique:
received:
09
02
2021
accepted:
09
02
2021
entrez:
22
8
2022
pubmed:
23
8
2022
medline:
23
8
2022
Statut:
epublish
Résumé
Autoimmune liver disease (AILD) spans a spectrum of chronic disorders affecting the liver parenchyma and biliary system. Three main categories of AILD are autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). This review condenses the presentation and discussions of the Single Topic Conference (STC) on AILD that was held in Ottawa, Ontario, in November 2019. We cover generalities regarding disease presentation and clinical diagnosis; mechanistic themes; treatment paradigms; clinical trials, including approaches and challenges to new therapies; and looking beyond traditional disease boundaries. Although these diseases are considered autoimmune, the etiology and role of environmental triggers are poorly understood. AILDs are progressive and chronic conditions that affect survival and quality of life. Advances have been made in PBC treatment because second-line treatments are now available (obeticholic acid, bezafibrate); however, a significant proportion still present suboptimal response. AIH treatment has remained unchanged for several decades, and data suggest that fewer than 50% of patients achieve a complete response and as many as 80% develop treatment-related side effects. B-cell depletion therapy to treat AIH is in an early stage of development and has shown promising results. An effective treatment for PSC is urgently needed. Liver transplant remains the best option for patients who develop decompensated cirrhosis or hepatocellular carcinoma within specific criteria, but recurrent AILD might occur. Continued efforts are warranted to develop networks for AILD aimed at assessing geo-epidemiological, clinical, and biochemical differences to capture the new treatment era in Canada.
Identifiants
pubmed: 35989897
doi: 10.3138/canlivj-2021-0006
pmc: PMC9235119
doi:
Types de publication
Journal Article
Langues
eng
Pagination
401-425Subventions
Organisme : Medical Research Council
ID : MR/L001489/1
Pays : United Kingdom
Informations de copyright
Copyright © 2021 Canadian Association for the Study of the Liver.
Déclaration de conflit d'intérêts
The authors have nothing to disclose.
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