Single Topic Conference on Autoimmune Liver Disease from the Canadian Association for the Study of the Liver
- 10 June 2021
- journal article
- research article
- Published by University of Toronto Press Inc. (UTPress) in Canadian Liver Journal
- Vol. 4 (4), 401-425
- https://doi.org/10.3138/canlivj-2021-0006
Abstract
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) in 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.Keywords
This publication has 125 references indexed in Scilit:
- Long-term medical management of the pediatric patient after liver transplantation: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of TransplantationLiver Transplantation, 2013
- Gut microbiota: next frontier in understanding human health and development of biotherapeuticsBiologics: Targets and Therapy, 2011
- Human leukocyte antigen in primary biliary cirrhosis: An old story now revivingJournal of Hepatology, 2011
- Incidence of primary sclerosing cholangitis: A systematic review and meta-analysisJournal of Hepatology, 2011
- Genome-wide association study identifies 12 new susceptibility loci for primary biliary cirrhosisNature Genetics, 2011
- Primary Biliary Cirrhosis Associated withHLA, IL12A,andIL12RB2VariantsThe New England Journal of Medicine, 2009
- Human leukocyte antigen polymorphisms in italian primary biliary cirrhosis: A multicenter study of 664 patients and 1992 healthy controlsJournal of Hepatology, 2008
- Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitisJournal of Hepatology, 2008
- The Value of Serum CA 19-9 in Predicting Cholangiocarcinomas in Patients with Primary Sclerosing CholangitisDigestive Diseases and Sciences, 2005
- Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring systemJournal of Hepatology, 2000