Cited by 114Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsDrug Transport and Resistance Mechanisms

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The challenges of primary biliary cholangitis: What is new and what needs to be done

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Terziroli Beretta-Piccoli, Benedetta, Mieli-Vergani, Giorgina, Vergani, Diego, Vierling, John M., Adams, David, Alpini, Gianfranco, Banales, Jesus M., Beuers, Ulrich, Björnsson, Einar, Bowlus, Christopher, Carbone, Marco, Chazouillères, Olivier, Dalekos, George, De Gottardi, Andrea, Harada, Kenichi, Hirschfield, Gideon, Invernizzi, Pietro, Jones, David, Krawitt, Edward, Lanzavecchia, Antonio, Lian, Zhe-Xiong, Ma, Xiong, Manns, Michael, Mavilio, Domenico, Quigley, Eamon MM., Sallusto, Federica, Shimoda, Shinji, Strazzabosco, Mario, Swain, Mark, Tanaka, Atsushi, Trauner, Michael, Tsuneyama, Koichi, Zigmond, Ehud, Gershwin, M. Eric·2019·Journal of Autoimmunity

DOI 10.1016/j.jaut.2019.102328

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Abstract

Primary Biliary Cholangitis (PBC) is an uncommon, chronic, cholangiopathy of autoimmune origin and unknown etiology characterized by positive anti-mitochondrial autoantibodies (AMA), female preponderance and progression to cirrhosis if left untreated. The diagnosis is based on AMA- or PBC-specific anti-nuclear antibody (ANA)-positivity in the presence of a cholestatic biochemical profile, histologic confirmation being mandatory only in seronegative cases. First-line treatment is ursodeoxycholic acid (UDCA), which is effective in preventing disease progression in about two thirds of the patients. The only approved second-line treatment is obeticholic acid. This article summarizes the most relevant conclusions of a meeting held in Lugano, Switzerland, from September 23rd-25th 2018, gathering basic and clinical scientists with various background from around the world to discuss the latest advances in PBC research. The meeting was dedicated to Ian Mackay, pioneer in the field of autoimmune liver diseases. The role of liver histology needs to be reconsidered: liver pathology consistent with PBC in AMA-positive individuals without biochemical cholestasis is increasingly reported, raising the question as to whether biochemical cholestasis is a reliable disease marker for both clinical practice and trials. The urgent need for new biomarkers, including more accurate markers of cholestasis, was also widely discussed during the meeting. Moreover, new insights in interactions of bile acids with biliary epithelia in PBC provide solid evidence of a role for impaired epithelial protection against potentially toxic hydrophobic bile acids, raising the fundamental question as to whether this bile acid-induced epithelial damage is the cause or the consequence of the autoimmune attack to the biliary epithelium. Strategies are needed to identify difficult-to-treat patients at an early disease stage, when new therapeutic approaches targeting immunologic pathways, in addition to bile acid-based therapies, may be effective. In conclusion, using interdisciplinary approaches, groundbreaking advances can be expected before long in respect to our understanding of the etiopathogenesis of PBC, with the ultimate aim of improving its treatment.

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Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients
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Genome-wide Association Study Identifies TNFSF15 and POU2AF1 as Susceptibility Loci for Primary Biliary Cirrhosis in the Japanese Population
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Emerging roles of Notch signaling in liver disease
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Identification and precursor frequency analysis of a common T cell epitope motif in mitochondrial autoantigens in primary biliary cirrhosis.
Shimoda, S., de Water, J. V., Ansari, A. A., Nakamura, M., Ishibashi, H., Coppel, R. L., Lake, J. R., Keeffe, E. B., Roche, T. E., & Gershwin, M. E. (1998) · Journal of Clinical Investigation · doi:10.1172/jci4213 · Cited by 250
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Up-regulation of microRNA 506 leads to decreased Cl−/HCO3 − anion exchanger 2 expression in biliary epithelium of patients with primary biliary cirrhosis
Banales, J. M., Sáez, E., Úriz, M., Sarvide, S., Urribarri, A. D., Splinter, P., Tietz Bogert, P. S., Bujanda, L., Prieto, J., Medina, J. F., et al. (2012) · Hepatology · doi:10.1002/hep.25691 · Cited by 250
Anti-Mitochondrial Antibodies and Primary Biliary Cirrhosis in TGF-β Receptor II Dominant-Negative Mice
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Bile acids and intestinal microbiota in autoimmune cholestatic liver diseases
Li, Y., Tang, R., Leung, P. S. C., Gershwin, M. E., & Ma, X. (2017) · Autoimmunity Reviews · doi:10.1016/j.autrev.2017.07.002 · Cited by 247
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Human liver-resident CD56bright/CD16neg NK cells are retained within hepatic sinusoids via the engagement of CCR5 and CXCR6 pathways
Hudspeth, K., Donadon, M., Cimino, M., Pontarini, E., Tentorio, P., Preti, M., Hong, M., Bertoletti, A., Bicciato, S., Invernizzi, P., et al. (2016) · Journal of Autoimmunity · doi:10.1016/j.jaut.2015.08.011 · Cited by 246
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Primary immunodeficiency and autoimmunity: A comprehensive review
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The microbiome and autoimmunity: a paradigm from the gut–liver axis
Li, B., Selmi, C. F., Tang, R., Gershwin, M. E., & Ma, X. (2018) · Cellular and Molecular Immunology · doi:10.1038/cmi.2018.7 · Cited by 237
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IL-2 receptor α−/− mice and the development of primary biliary cirrhosis
Wakabayashi, K., Lian, Z.-X., Moritoki, Y., Lan, R. Y., Tsuneyama, K., Chuang, Y.-H., Yang, G.-X., Ridgway, W., Ueno, Y., Ansari, A. A., et al. (2006) · Hepatology · doi:10.1002/hep.21385 · Cited by 233
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Ursodeoxycholic acid and bile-acid mimetics as therapeutic agents for cholestatic liver diseases: An overview of their mechanisms of action
Poupon, R. Y. (2012) · Clinics and Research in Hepatology and Gastroenterology · doi:10.1016/s2210-7401(12)70015-3 · Cited by 230
Positive antimitochondrial antibody but normal alkaline phosphatase: Is this primary biliary cirrhosis?
Mitchison, H. C., Bassendine, M. F., Hendrick, A., Bennett, M. K., Bird, G., Watson, A. J., & James, O. F. W. (1986) · Hepatology · doi:10.1002/hep.1840060609 · Cited by 230
Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study
Mells, G. F., Pells, G., Newton, J. L., Bathgate, A. J., Burroughs, A. K., Heneghan, M. A., Neuberger, J. M., Day, D. B., Ducker, S. J., Sandford, R. N., et al. (2013) · Hepatology · doi:10.1002/hep.26365 · Cited by 227
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New therapeutic concepts in bile acid transport and signaling for management of cholestasis
Trauner, M., Fuchs, C. D., Halilbasic, E., & Paumgartner, G. (2017) · Hepatology · doi:10.1002/hep.28991 · Cited by 227
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Bile Salts Control the Antimicrobial Peptide Cathelicidin Through Nuclear Receptors in the Human Biliary Epithelium
d'Aldebert, E., Mve, M.-J. B. B., Mergey, M., Wendum, D., Firrincieli, D., Coilly, A., Fouassier, L., Corpechot, C., Poupon, R. Y., Housset, C., et al. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.12.040 · Cited by 225
Application of a new histological staging and grading system for primary biliary cirrhosis to liver biopsy specimens: Interobserver agreement
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Apotopes and the biliary specificity of primary biliary cirrhosis†
Lleo, A., Selmi, C., Invernizzi, P., Podda, M., Coppel, R. L., Mackay, I. R., Gores, G. J., Ansari, A. A., Van de Water, J., & Gershwin, M. E. (2009) · Hepatology · doi:10.1002/hep.22736 · Cited by 222
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Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: A three‐year randomized trial†
Rautiainen, H., Kärkkäinen, P., Karvonen, A., Nurmi, H., Pikkarainen, P., Nuutinen, H., & Färkkilä, M. (2005) · Hepatology · doi:10.1002/hep.20646 · Cited by 222
IL-12/Th1 and IL-23/Th17 Biliary Microenvironment in Primary Biliary Cirrhosis: Implications for Therapy
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Biliary epithelium and liver B cells exposed to bacteria activate intrahepatic MAIT cells through MR1
Jeffery, H. C., van Wilgenburg, B., Kurioka, A., Parekh, K., Stirling, K., Roberts, S., Dutton, E. E., Hunter, S., Geh, D., Braitch, M. K., et al. (2016) · Journal of Hepatology · doi:10.1016/j.jhep.2015.12.017 · Cited by 212
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Primary biliary cirrhosis is associated with altered hepatic microRNA expression
Padgett, K. A., Lan, R. Y., Leung, P. C., Lleò, A., Dawson, K., Pfeiff, J., Mao, T. K., Coppel, R. L., Ansari, A. A., & Gershwin, M. E. (2009) · Journal of Autoimmunity · doi:10.1016/j.jaut.2009.02.022 · Cited by 209
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Route of Antigen Uptake Differentially Impacts Presentation by Dendritic Cells and Activated Monocytes
Kamphorst, A. O., Guermonprez, P., Dudziak, D., & Nussenzweig, M. C. (2010) · The Journal of Immunology · doi:10.4049/jimmunol.1001205 · Cited by 208
Decreased Anion Exchanger 2 Immunoreactivity in the Liver of Patients With Primary Biliary Cirrhosis
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Hepatic IL-17 responses in human and murine primary biliary cirrhosis
Lan, R. Y. Z., Salunga, T. L., Tsuneyama, K., Lian, Z.-X., Yang, G.-X., Hsu, W., Moritoki, Y., Ansari, A. A., Kemper, C., Price, J., et al. (2009) · Journal of Autoimmunity · doi:10.1016/j.jaut.2008.11.001 · Cited by 202
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Stromal Fibroblasts in Tertiary Lymphoid Structures: A Novel Target in Chronic Inflammation
Barone, F., Gardner, D. H., Nayar, S., Steinthal, N., Buckley, C. D., & Luther, S. A. (2016) · Frontiers in Immunology · doi:10.3389/fimmu.2016.00477 · Cited by 199
A controlled trial of prednisolone treatment in primary biliary cirrhosis
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Role of nuclear bile acid receptor, FXR, in adaptive ABC transporter regulation by cholic and ursodeoxycholic acid in mouse liver, kidney and intestine
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Ae2-Deficient Mice Develop Antimitochondrial Antibodies and Other Features Resembling Primary Biliary Cirrhosis
Salas, J. T., Bañales, J. M., Sarvide, S., Recalde, S., Ferrer, A., Uriarte, I., Elferink, R. P. J. O., Prìeto, J., & Medina, J. F. (2008) · Gastroenterology · doi:10.1053/j.gastro.2008.02.020 · Cited by 196
Development of the bile ducts: Essentials for the clinical hepatologist
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Altered Monocyte Responses to Defined TLR Ligands in Patients With Primary Biliary Cirrhosis *
Mao, T. K., Lian, Z.-X., Selmi, C., Ichiki, Y., Ashwood, P., Ansari, A. A., Coppel, R. L., Shimoda, S., Ishibashi, H., & Gershwin, E. M. (2005) · Hepatology · doi:10.1002/hep.20859 · Cited by 185
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The Nuclear Receptor PPARs as Important Regulators of T-Cell Functions and Autoimmune Diseases
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Macrophages contribute to the pathogenesis of sclerosing cholangitis in mice
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Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease†
Wakabayashi, K., Lian, Z., Leung, P. S. C., Moritoki, Y., Tsuneyama, K., Kurth, M. J., Lam, K. S., Yoshida, K., Yang, G., Hibi∥, T., et al. (2008) · Hepatology · doi:10.1002/hep.22390 · Cited by 180
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Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study
Trauner, M., Nevens, F., Shiffman, M. L., Drenth, J. P. H., Bowlus, C. L., Vargas, V., Andreone, P., Hirschfield, G. M., Pencek, R., Malecha, E. S., et al. (2019) · The Lancet Gastroenterology & Hepatology · doi:10.1016/s2468-1253(19)30094-9 · Cited by 180
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The Surface-Associated Exopolysaccharide of Bifidobacterium longum 35624 Plays an Essential Role in Dampening Host Proinflammatory Responses and Repressing Local T H 17 Responses
Schiavi, E., Gleinser, M., Molloy, E. M., Groeger, D., Frei, R., Ferstl, R., Rodríguez‐Pérez, N., Ziegler, M., Grant, R., Moriarty, T. F., et al. (2016) · Applied and Environmental Microbiology · doi:10.1128/aem.02238-16 · Cited by 179
NOD.c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis
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The FXR agonist PX20606 ameliorates portal hypertension by targeting vascular remodelling and sinusoidal dysfunction
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Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis
Wesierska-Gadek, J., Penner, E., Battezzati, P. M., Selmi, C., Zuin, M., Hitchman, E., Worman, H. J., Gershwin, E. M., Podda, M., & Invernizzi, P. (2006) · Hepatology · doi:10.1002/hep.21172 · Cited by 177
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Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle upon Tyne, England
Metcalf, J. V., Bhopal, R. S., Gray, J. W., Howel, D., & James, O. (1997) · International Journal of Epidemiology · doi:10.1093/ije/26.4.830 · Cited by 176
Pemphigus autoantibodies generated through somatic mutations target the desmoglein-3 cis-interface
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Comprehensive Analysis of Serum and Fecal Bile Acid Profiles and Interaction with Gut Microbiota in Primary Biliary Cholangitis
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Changing nomenclature for PBC: From ‘cirrhosis’ to ‘cholangitis’
Beuers, U. H., Gershwin, M. E., Gish, R. G., Invernizzi, P., Jones, D. E., Lindor, K. D., Ma, X., Mackay, I. R., Parés, A. R., Tanaka, A., et al. (2015) · Hepatology · doi:10.1002/hep.28140 · Cited by 165
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Overlap of Autoimmune Hepatitis and Primary Biliary Cirrhosis: Long-Term Outcomes
Silveira, M. G., Talwalkar, J. A., Angulo, P., & Lindor, K. D. (2007) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.2007.01136.x · Cited by 165
Pharmacokinetics and Pharmacodynamic Action of Budesonide in Early– and Late–Stage Primary Biliary Cirrhosis
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Changes in microbial ecology after fecal microbiota transplantation for recurrent C. difficile infection affected by underlying inflammatory bowel disease
Khanna, S., Vázquez‐Baeza, Y., González, A., Weiss, S. J., Schmidt, B. A., Pedrogo, D. A. M., Rainey, J. F., Kammer, P. P., Nelson, H., Sadowsky, M. J., et al. (2017) · Microbiome · doi:10.1186/s40168-017-0269-3 · Cited by 163
Hepatic Expression of Secondary Lymphoid Chemokine (CCL21) Promotes the Development of Portal-Associated Lymphoid Tissue in Chronic Inflammatory Liver Disease
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Fractalkine and CX3CR1 are involved in the recruitment of intraepithelial lymphocytes of intrahepatic bile ducts†
Isse, K., Harada, K., Zen, Y., Kamihira, T., Shimoda, S., Harada, M., & Nakanuma, Y. (2005) · Hepatology · doi:10.1002/hep.20582 · Cited by 157
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Cognitive impairment in primary biliary cirrhosis: Symptom impact and potential etiology†
Newton, J. L., Hollingsworth, K. G., Taylor, R., El‐Sharkawy, A. M., Khan, Z. U., Pearce, R., Sutcliffe, K., Okonkwo, O., Davidson, A., Burt, J., et al. (2008) · Hepatology · doi:10.1002/hep.22371 · Cited by 156
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Pretreatment prediction of response to ursodeoxycholic acid in primary biliary cholangitis: development and validation of the UDCA Response Score
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Biochemical and Immunologic Effects of Rituximab in Patients With Primary Biliary Cirrhosis and an Incomplete Response to Ursodeoxycholic Acid
Tsuda, M., Moritoki, Y., Lian, Z.-X., Zhang, W., Yoshida, K., Wakabayashi, K., Yang, G., Nakatani, T., Vierling, J. M., Lindor, K. D., et al. (2011) · Hepatology · doi:10.1002/hep.24748 · Cited by 151
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Immunochip analyses identify a novel risk locus for primary biliary cirrhosis at 13q14, multiple independent associations at four established risk loci and epistasis between 1p31 and 7q32 risk variants
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Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long‐term outcome and survival
Al‐Chalabi, T., Portmann, B. C., Bernal, W., McFarlane, I. G., & Heneghan, M. A. (2008) · Alimentary Pharmacology & Therapeutics · doi:10.1111/j.1365-2036.2008.03722.x · Cited by 149
Primary Biliary Cirrhosis Showing a High Titer of Autoantibody
Mackay, I. R. (1958) · New England Journal of Medicine · doi:10.1056/nejm195801232580407 · Cited by 147
Transplantation trends in primary biliary cirrhosis
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Effects of angiogenic factor overexpression by human and rodent cholangiocytes in polycystic liver diseases
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Development of autoantibodies precedes clinical manifestations of autoimmune diseases: A comprehensive review
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Nuclear receptors as therapeutic targets in cholestatic liver diseases
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Bacterial CpG induces hyper-IgM production in CD27+ memory B cells in primary biliary cirrhosis
Kikuchi, K., Lian, Z., Yang, G., Ansari, A. A., Ikehara, S., Kaplan, M., Miyakawa, H., Coppel, R. L., & Gershwin, M. E. (2005) · Gastroenterology · doi:10.1053/j.gastro.2004.11.005 · Cited by 141
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A genome-wide association study identifies six novel risk loci for primary biliary cholangitis
Qiu, F., Tang, R., Zuo, X., Shi, X., Wei, Y., Zheng, X., Dai, Y., Gong, Y., Wang, L., Xu, P., et al. (2017) · Nature Communications · doi:10.1038/ncomms14828 · Cited by 140
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Combined ursodeoxycholic acid (UDCA) and fenofibrate in primary biliary cholangitis patients with incomplete UDCA response may improve outcomes
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Increasing Prevalence of Primary Biliary Cholangitis and Reduced Mortality With Treatment
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Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis
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Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice
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Assessment of biliary bicarbonate secretion in humans by positron emission tomography
Prìeto, J., García, N., Martí-Climent, J. M., Peñuelas, I., Richter, J. Á., & Medina, J. F. (1999) · Gastroenterology · doi:10.1016/s0016-5085(99)70564-0 · Cited by 135
Factors Associated With Recurrence of Primary Biliary Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival
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Ustekinumab for patients with primary biliary cholangitis who have an inadequate response to ursodeoxycholic acid: A proof‐of‐concept study
Hirschfield, G. M., Gershwin, M. E., Strauss, R. S., Mayo, M. J., Levy, C., Zou, B., Johanns, J., Nnane, I. P., Dasgupta, B., Li, K. T., et al. (2015) · Hepatology · doi:10.1002/hep.28359 · Cited by 131
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THE RELATIONSHIP BETWEEN SERUM LIPIDS AND SKIN XANTHOMATA IN EIGHTEEN PATIENTS WITH PRIMARY BILIARY CIRRHOSIS
AHRENS, E. H., & Kunkel, H. G. (1949) · Journal of Clinical Investigation · doi:10.1172/jci102222 · Cited by 129
Systematic review: recurrent autoimmune liver diseases after liver transplantation
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Interaction between Toll-like receptors and natural killer cells in the destruction of bile ducts in primary biliary cirrhosis
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Fatigue in chronic liver disease: New insights and therapeutic approaches
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B-Cell Depletion With Rituximab in Patients With Primary Biliary Cirrhosis Refractory to Ursodeoxycholic Acid
Myers, R. P., Swain, M. G., Lee, S. S., Shaheen, A. A., & Burak, K. W. (2013) · The American Journal of Gastroenterology · doi:10.1038/ajg.2013.51 · Cited by 124
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Chronic expression of interferon‐gamma leads to murine autoimmune cholangitis with a female predominance
Bae, H. R., Leung, P. S. C., Tsuneyama, K., Valencia, J. C., Hodge, D. L., Kim, S., Back, T., Karwan, M., Merchant, A. S., Baba, N., et al. (2016) · Hepatology · doi:10.1002/hep.28641 · Cited by 123
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IL-10-producing regulatory B cells restrain the T follicular helper cell response in primary Sjögren’s syndrome
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Authors: Terziroli Beretta-Piccoli, B., Mieli-Vergani, G., Vergani, D., Vierling, J. M., Adams, D., Alpini, G., Banales, J. M., Beuers, U., Björnsson, E., Bowlus, C., Carbone, M., Chazouillères, O., Dalekos, G., De Gottardi, A., Harada, K., Hirschfield, G., Invernizzi, P., Jones, D., Krawitt, E., Lanzavecchia, A., Lian, Z.-X., Ma, X., Manns, M., Mavilio, D., Quigley, E. M., Sallusto, F., Shimoda, S., Strazzabosco, M., Swain, M., Tanaka, A., Trauner, M., Tsuneyama, K., Zigmond, E., & Gershwin, M. E.
Affiliations: King's College Hospital (GB)
Baylor College of Medicine (US)
NIHR Birmingham Biomedical Research Centre (GB)
Richard L. Roudebush VA Medical Center (US)
Amsterdam UMC Location University of Amsterdam (NL)
University of California, Davis (US)
University of Milan (IT)
Hôpital Saint-Antoine (FR)
University of Thessaly (GR)
Kanazawa University (JP)
University Health Network (CA)
NIHR Newcastle Biomedical Research Centre (GB)
University of Vermont (US)
Institute for Research in Biomedicine (CH)
South China University of Technology (CN)
Shanghai Jiao Tong University (CN)
Medizinische Hochschule Hannover (DE)
Houston Methodist (US)
Kyushu University (JP)
Yale University (US)
University of Calgary (CA)
Teikyo University (JP)
Medical University of Vienna (AT)
Tokushima University (JP)
Tel Aviv University (IL)
ORCID: Vergani, Diego
Vierling, John M.
Adams, David
Alpini, Gianfranco
Beuers, Ulrich
Bowlus, Christopher
Carbone, Marco
Chazouillères, Olivier
Dalekos, George
Harada, Kenichi
Hirschfield, Gideon
Invernizzi, Pietro
Jones, David
Lanzavecchia, Antonio
Lian, Zhe-Xiong
Ma, Xiong
Manns, Michael
Mavilio, Domenico
Sallusto, Federica
Shimoda, Shinji
Strazzabosco, Mario
Swain, Mark
Tanaka, Atsushi
Trauner, Michael
Tsuneyama, Koichi
Zigmond, Ehud
Gershwin, M. Eric
Venue: Journal of Autoimmunity
Publisher: Elsevier BV
Published: 2019-12
Volume: 105
Pages: 102328
ISSN: 0896-8411 (ISSN-L)
DOI: 10.1016/j.jaut.2019.102328
OpenAlex: W2974241762
Cited by: 114
References: 318
Citations by year: 2026: 2 · 2025: 13 · 2024: 13 · 2023: 18 · 2022: 26 · 2021: 21 · 2020: 18 · 2019: 3
FWCI: 2.23
Type: journal article
Language: en
Open access: green
research.birmingham.ac.uk
Licence: cc-by-nc-nd
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: Journal of Autoimmunity (publishedVersion)
PubMed (publishedVersion)
Data Archiving and Networked Services (DANS) (submittedVersion)
BOA (University of Milano-Bicocca) (submittedVersion)
University of Thessaly Institutional Repository (University of Thessaly) (submittedVersion)
Institutional Repositories DataBase (IRDB) (submittedVersion)
Pure Amsterdam UMC (submittedVersion)
University of Birmingham Research Portal (University of Birmingham) (submittedVersion, cc-by-nc-nd)
Hirsla (Landspítali University Hospital) (submittedVersion)
Funders: National Institute for Health and Care Research (NIHR200243, NF-SI-0513-10091)
Medical Research Council (MR/L001489/1)
Japan Society for the Promotion of Science (18K07069, 17H04058)
Topics: Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
Keywords: primary biliary cirrhosis, antimitochondrial antibodies, antinuclear antibodies, ursodeoxycholic acid, obeticholic acid, liver histopathology, bile acids, biliary epithelium, autoimmune liver disease, disease progression, difficult-to-treat patients, etiopathogenesis
MeSH terms: Antibodies, Antinuclear, Autoimmune Diseases, Chenodeoxycholic Acid, Cholagogues and Choleretics, Congresses as Topic, Female, Humans, Liver, Liver Cirrhosis, Biliary, Ursodeoxycholic Acid

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The challenges of primary biliary cholangitis: What is new and what needs to be done

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