Lleo A, Wang GQ, Gershwin ME, Hirschfield GM. Primary biliary cholangitis. Lancet. 2020;396:1915–26. https://doi.org/10.1016/S0140-6736(20)31607-X.

Article  PubMed  CAS  Google Scholar 

Lleo A, Jepsen P, Morenghi E, Carbone M, Moroni L, Battezzati PM, et al. Evolving trends in female to male incidence and male mortality of primary biliary cholangitis. Sci Rep. 2016;6:25906. https://doi.org/10.1038/srep25906.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Tanaka A, Mori M, Matsumoto K, Ohira H, Tazuma S, Takikawa H. Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res. 2019;49:881–9. https://doi.org/10.1111/hepr.13342.

Article  PubMed  CAS  Google Scholar 

Lleo A, Marzorati S, Anaya JM, Gershwin ME. Primary biliary cholangitis: a comprehensive overview. Hepatol Int. 2017;11:485–99. https://doi.org/10.1007/s12072-017-9830-1.

Article  PubMed  Google Scholar 

Poupon RE, Balkau B, Eschwège E, Poupon R. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC study group. N Engl J Med. 1991;324:1548–54. https://doi.org/10.1056/NEJM199105303242204.

Article  PubMed  CAS  Google Scholar 

Poupon RE, Eschwège E, Poupon R. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. Interim analysis of a double-blind multicentre randomized trial. The UDCA-PBC study group. J Hepatol. 1990;11:16–21. https://doi.org/10.1016/0168-8278(90)90265-s.

Article  PubMed  CAS  Google Scholar 

Beuers U, Gershwin ME, Gish RG, Invernizzi P, Jones DE, Lindor K, et al. Changing nomenclature for PBC: from “cirrhosis” to “cholangitis.” J Hepatol. 2015;63:1285–7. https://doi.org/10.1016/j.jhep.2015.06.031.

Article  PubMed  Google Scholar 

European Association for the Study of the Liver. Electronic address: easloffice@easloffice.eu, European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67:145–72. https://doi.org/10.1016/j.jhep.2017.03.022

Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69:394–419. https://doi.org/10.1002/hep.30145

Nevens F, Andreone P, Mazzella G, Strasser SI, Bowlus C, Invernizzi P, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med. 2016;375:631–43. https://doi.org/10.1056/NEJMoa1509840.

Article  PubMed  CAS  Google Scholar 

Corpechot C, Chazouillères O, Rousseau A. Bezafibrate in primary biliary cholangitis. N Engl J Med. 2018;379:985. https://doi.org/10.1056/NEJMc1809061.

Article  PubMed  Google Scholar 

Tanaka A, Hirohara J, Nakano T, Matsumoto K, Chazouillères O, Takikawa H, et al. Association of bezafibrate with transplant-free survival in patients with primary biliary cholangitis. J Hepatol. 2021;75:565–71. https://doi.org/10.1016/j.jhep.2021.04.010.

Article  PubMed  CAS  Google Scholar 

Lleo A, Bowlus CL, Yang GX, Invernizzi P, Podda M, Van de Water J, et al. Biliary apotopes and anti-mitochondrial antibodies activate innate immune responses in primary biliary cirrhosis. Hepatology. 2010;52:987–98. https://doi.org/10.1002/hep.23783.

Article  PubMed  CAS  Google Scholar 

Lleo A, Selmi C, Invernizzi P, Podda M, Coppel RL, Mackay IR, et al. Apotopes and the biliary specificity of primary biliary cirrhosis. Hepatology. 2009;49:871–9. https://doi.org/10.1002/hep.22736.

Article  PubMed  CAS  Google Scholar 

Cordell HJ, Fryett JJ, Ueno K, Darlay R, Aiba Y, Hitomi Y, et al. An international genome-wide meta-analysis of primary biliary cholangitis: novel risk loci and candidate drugs. J Hepatol. 2021;75:572–81. https://doi.org/10.1016/j.jhep.2021.04.055.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Beuers U, Hohenester S, de Buy Wenniger LJ, Kremer AE, Jansen PL, Elferink RP. The biliary HCO(3)(−) umbrella: a unifying hypothesis on pathogenetic and therapeutic aspects of fibrosing cholangiopathies. Hepatology. 2010;52:1489–96. https://doi.org/10.1002/hep.23810.

Article  PubMed  CAS  Google Scholar 

Banales JM, Sáez E, Uriz M, Sarvide S, Urribarri AD, Splinter P, et al. Up-regulation of microRNA 506 leads to decreased Cl−/HCO3− anion exchanger 2 expression in biliary epithelium of patients with primary biliary cirrhosis. Hepatology. 2012;56:687–97. https://doi.org/10.1002/hep.25691.

Article  PubMed  CAS  Google Scholar 

Asselta R, Paraboschi EM, Gerussi A, Cordell HJ, Mells GF, Sandford RN, et al. X chromosome contribution to the genetic architecture of primary biliary cholangitis. Gastroenterology. 2021;160:2483-2495.e26. https://doi.org/10.1053/j.gastro.2021.02.061.

Article  PubMed  CAS  Google Scholar 

Poupon RE, Chrétien Y, Chazouillères O, Poupon R, Chwalow J. Quality of life in patients with primary biliary cirrhosis. Hepatology. 2004;40:489–94. https://doi.org/10.1002/hep.20276.

Article  PubMed  Google Scholar 

Newton JL, Bhala N, Burt J, Jones DE. Characterisation of the associations and impact of symptoms in primary biliary cirrhosis using a disease specific quality of life measure. J Hepatol. 2006;44:776–83. https://doi.org/10.1016/j.jhep.2005.12.012.

Article  PubMed  Google Scholar 

Selmi C, Gershwin ME, Lindor KD, Worman HJ, Gold EB, Watnik M, et al. Quality of life and everyday activities in patients with primary biliary cirrhosis. Hepatology. 2007;46:1836–43. https://doi.org/10.1002/hep.21953.

Article  PubMed  Google Scholar 

Mells GF, Pells G, Newton JL, Bathgate AJ, Burroughs AK, Heneghan MA, et al. Impact of primary biliary cirrhosis on perceived quality of life: the UK-PBC national study. Hepatology. 2013;58:273–83. https://doi.org/10.1002/hep.26365.

Article  PubMed  Google Scholar 

Dyson JK, Wilkinson N, Jopson L, Mells G, Bathgate A, Heneghan MA, et al. The inter-relationship of symptom severity and quality of life in 2055 patients with primary biliary cholangitis. Aliment Pharmacol Ther. 2016;44:1039–50. https://doi.org/10.1111/apt.13794.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Yagi M, Tanaka A, Abe M, Namisaki T, Yoshiji H, Takahashi A, et al. Symptoms and health-related quality of life in Japanese patients with primary biliary cholangitis. Sci Rep. 2018;8:12542. https://doi.org/10.1038/s41598-018-31063-8.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Beuers U, Wolters F, Oude Elferink RPJ. Mechanisms of pruritus in cholestasis: understanding and treating the itch. Nat Rev Gastroenterol Hepatol. 2023;20:26–36. https://doi.org/10.1038/s41575-022-00687-7.

Article  PubMed  Google Scholar 

Düll MM, Kremer AE. Evaluation and management of pruritus in primary biliary cholangitis. Clin Liver Dis. 2022;26:727–45. https://doi.org/10.1016/j.cld.2022.06.009.

Article  PubMed  Google Scholar 

Lynch EN, Campani C, Innocenti T, Dragoni G, Biagini MR, Forte P, et al. Understanding fatigue in primary biliary cholangitis: from pathophysiology to treatment perspectives. World J Hepatol. 2022;14:1111–9. https://doi.org/10.4254/wjh.v14.i6.1111.

Article  PubMed  PubMed Central  Google Scholar 

Phaw NA, Leighton J, Dyson JK, Jones DE. Managing cognitive symptoms and fatigue in cholestatic liver disease. Expert Rev Gastroenterol Hepatol. 2021;15:235–41. https://doi.org/10.1080/17474124.2021.1844565.

Article  PubMed  CAS  Google Scholar 

Harms MH, van Buuren HR, Corpechot C, Thorburn D, Janssen HLA, Lindor KD, et al. Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. J Hepatol. 2019;71:357–65. https://doi.org/10.1016/j.jhep.2019.04.001.

Article  PubMed  CAS  Google Scholar 

Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019;69:394–419. https://doi.org/10.1002/hep.30145.

Article  PubMed  Google Scholar 

Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases. Hepatology. 2022;75:1012–3. https://doi.org/10.1002/hep.32117.

Article  PubMed  Google Scholar 

John BV, Schwartz K, Levy C, et al. Impact of obeticholic acid exposure on decompensation and mortality in primary biliary cholangitis and cirrhosis. Hepatol Commun. 2021;5:1426–36. https://doi.org/10.1002/hep4.1720.

Article  PubMed  PubMed Central  CAS  Google Scholar 

De Vincentis A, D’Amato D, Cristoferi L, et al. Predictors of serious adverse events and non-response in cirrhotic patients with primary biliary cholangitis treated with obeticholic acid. Liver Int. 2022;42:2453–65. https://doi.org/10.1111/liv.15386.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Roberts SB, Ismail M, Kanagalingam G, et al. Real-world effectiveness of obeticholic acid in patients with primary biliary cholangitis. Hepatol Commun. 2020;4:1332–45. https://doi.org/10.1002/hep4.1518.

Article  PubMed  PubMed Central  CAS  Google Scholar 

D’Amato D, De Vincentis A, Malinverno F, et al. Real-world experience with obeticholic acid in patients with primary biliary cholangitis. JHEP Rep (Online). 2021;3: 100248.