In 2016, obeticholic acid became the first new licensed therapy for primary biliary cholangitis in >20 years. This therapeutic came at a time of improved disease understanding from biliary and immunological mechanistic insights.
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References
Hirschfield, G. M. & Gershwin, M. E. The immunobiology and pathophysiology of primary biliary cirrhosis. Annu. Rev. Pathol. 8, 303–330 (2013).
Trivedi, P. J., Corpechot, C., Pares, A. & Hirschfield, G. M. Risk stratification in autoimmune cholestatic liver diseases: opportunities for clinicians and trialists. Hepatology 63, 644–659 (2016).
Bae, H. R. et al. Chronic expression of interferon-gamma leads to murine autoimmune cholangitis with a female predominance. Hepatology 64, 1189–1201 (2016).
Cordell, H. J. et al. International genome-wide meta-analysis identifies new primary biliary cirrhosis risk loci and targetable pathogenic pathways. Nat. Commun. 6, 8019 (2015).
Chang, J. C. et al. Soluble adenylyl cyclase regulates bile salt-induced apoptosis in human cholangiocytes. Hepatology 64, 522–534 (2016).
Hisamoto, S. et al. Hydrophobic bile acids suppress expression of AE2 in biliary epithelial cells and induce bile duct inflammation in primary biliary cholangitis. J. Autoimmun. 75, 150–160 (2016).
Oo, Y. H. et al. CXCR3-dependent recruitment and CCR6-mediated positioning of Th-17 cells in the inflamed liver. J. Hepatol. 57, 1044–1051 (2012).
Luo, J. et al. A nontumorigenic variant of FGF19 treats cholestatic liver diseases. Sci. Transl Med. 6, 247ra100 (2014).
Zhou, M. et al. Engineered fibroblast growth factor 19 reduces liver injury and resolves sclerosing cholangitis in Mdr2-deficient mice. Hepatology 63, 914–929 (2016).
Nevens, F. et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N. Engl. J. Med. 375, 631–643 (2016).
Acknowledgements
G.J.W. and G.M.H. are supported by the NIHR Birmingham Biomedical Research Unit. This report represents independent research partly supported by the NIHR Biomedical Research Unit Funding Scheme. The views expressed in this publication are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
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G.M.H. has been on primary biliary cholangitis advisory boards for GlaxoSmithKline, Intercept Pharmaceuticals and Novartis. G.M.H. has been a study investigator for CymaBay Therapeutics, Dr Falk Pharma, FF Pharma, GlaxoSmithKline, Gilead, Intercept Pharmaceuticals, NGM Bio, Novartis and Shire.
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Webb, G., Hirschfield, G. High-definition PBC: biology, models and therapeutic advances. Nat Rev Gastroenterol Hepatol 14, 76–78 (2017). https://doi.org/10.1038/nrgastro.2016.201
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DOI: https://doi.org/10.1038/nrgastro.2016.201
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