Sodium/bile acid co-transporter inhibitors currently in preclinical or early clinical development for the treatment of primary biliary cholangitis

References

• Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet [Internet]. 2015 [cited 2023 Dec 15];386(10003):1565–1575. Available from https://pubmed.ncbi.nlm.nih.gov/26364546/
   PubMed Web of Science ®Google Scholar
• Griffiths L, Dyson JK, Jones DEJ. The new epidemiology of primary biliary cirrhosis. Semin Liver Dis [Internet]. 2014 [cited 2023 Dec 15];34(03):318–328. Available from: https://pubmed.ncbi.nlm.nih.gov/25057954/
   PubMedGoogle Scholar
• Taylor R, Jacobs M, Phillips S ANNUAL REPORT on LIVER TRANSPLANTATION REPORT for 2022/2023 (1 APRIL 2013-31 MARCH 2023) [Internet]. [cited 2023 Dec 15]. Available from: https://nhsbtdbe.blob.core.windows.net/umbraco-assets-corp/30875/nhsbt-liver-transplantation-report-2223.pdf.
   Google Scholar
• Hegade VS, Mells GF, Fisher H, et al. Pruritus is common and undertreated in patients with primary biliary cholangitis in the United Kingdom. Clin Gastroenterol Hepatol [Internet]. 2019 [cited 2023 Dec 15];17(7):1379–1387.e3. Available from: https://pubmed.ncbi.nlm.nih.gov/30557739/
   PubMed Web of Science ®Google Scholar
• Mayo MJ, Carey E, Smith HT, et al. Impact of pruritus on quality of life and current treatment patterns in patients with primary biliary cholangitis. Dig Dis Sci [Internet]. 2023 [cited 2023 Dec 15];68:995–1005. Available from: https://pubmed.ncbi.nlm.nih.gov/35704252/
   PubMed Web of Science ®Google Scholar
• Abbas N, Culver EL, Thorburn D, et al. UK-Wide multicenter evaluation of second-line therapies in primary biliary cholangitis. Clin Gastroenterol Hepatol [Internet]. 2023 [cited 2023 Dec 15];21(6):1561–1570.e13. Available from: https://pubmed.ncbi.nlm.nih.gov/35961518/
   PubMed Web of Science ®Google Scholar
• Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid. Gastroenterology [Internet]. 2006 [cited 2024 Jan 24];130(3):715–720. Available from: https://pubmed.ncbi.nlm.nih.gov/16530513/
   PubMed Web of Science ®Google Scholar
• Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology [Internet]. 1997 [cited 2024 Jan 24];113(3):884–890. Available from: https://pubmed.ncbi.nlm.nih.gov/9287980/
   PubMed Web of Science ®Google Scholar
• Talwalkar JA, Souto E, Jorgensen RA, et al. Natural history of pruritus in primary biliary cirrhosis. Clin Gastroenterol Hepatol [Internet]. 2003 [cited 2023 Dec 15];1(4):297–302. Available from: http://www.cghjournal.org/article/S1542356503001344/fulltext
   PubMed Web of Science ®Google Scholar
• Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med [Internet]. 2016 [cited 2023 Dec 15];375(7):631–643. Available from: https://pubmed.ncbi.nlm.nih.gov/27532829/
   PubMed Web of Science ®Google Scholar
• Millson C, Considine A, Cramp ME, et al. Adult liver transplantation: a UK clinical guideline-part 1: pre-operation. Gastroenterology [Internet]. 2020 [cited 2023 Dec 15];1–10. Available from: https://www
   Web of Science ®Google Scholar
• Smith HT, de Souza AR, Thompson AH, et al. Cholestatic pruritus treatments in primary biliary cholangitis and primary sclerosing cholangitis: a systematic literature review. Dig Dis Sci [Internet]. 2023 [cited 2023 Dec 15];68(6):2710. Available from: /pmc/articles/PMC10024020/
   PubMed Web of Science ®Google Scholar
• Roberts SB, Ismail M, Kanagalingam G, et al. Real‐world effectiveness of obeticholic acid in patients with primary biliary cholangitis. Hepatol Commun [Internet]. 2020 [cited 2023 Dec 15];4(9):1332. Available from: /pmc/articles/PMC7471421/
   PubMed Web of Science ®Google Scholar
• Corpechot C, Chazouillères O, Rousseau A, et al. A placebo-controlled trial of bezafibrate in primary biliary cholangitis. N Engl J Med [Internet]. 2018 [cited 2023 Dec 15];378(23):2171–2181. Available from: https://pubmed.ncbi.nlm.nih.gov/29874528/
   PubMed Web of Science ®Google Scholar
• de Vries E, Bolier R, Goet J, et al. Fibrates for itch (FITCH) in fibrosing cholangiopathies: a double-blind, randomized, placebo-controlled trial. Gastroenterology [Internet]. 2021 [cited 2023 Dec 15];160(3):734–743.e6. Available from: https://pubmed.ncbi.nlm.nih.gov/33031833/
   PubMed Web of Science ®Google Scholar
• Schattenberg JM, Pares A, Kowdley KV, et al. A randomized placebo-controlled trial of elafibranor in patients with primary biliary cholangitis and incomplete response to UDCA. J Hepatol [Internet]. 2021 [cited 2023 Dec 15];74(6):1344–1354. Available from: https://pubmed.ncbi.nlm.nih.gov/33484775/
   PubMed Web of Science ®Google Scholar
• Kremer AE, Mayo MJ, Hirschfield G, et al. Seladelpar improved measures of pruritus, sleep, and fatigue and decreased serum bile acids in patients with primary biliary cholangitis. Liver Int [Internet]. 2022 [cited 2023 Dec 15];42(1):112–123. Available from: https://pubmed.ncbi.nlm.nih.gov/34403559/
   PubMed Web of Science ®Google Scholar
• Hirschfield GM, Shiffman ML, Gulamhusein A, et al. Seladelpar efficacy and safety at 3 months in patients with primary biliary cholangitis: ENHANCE, a phase 3, randomized, placebo-controlled study. Hepatology [Internet]. 2023 [cited 2023 Dec 15];78(2):397–415. Available from: https://pubmed.ncbi.nlm.nih.gov/37386786/
   PubMed Web of Science ®Google Scholar
• Ghent CN, Carruthers SG. Treatment of pruritus in primary biliary cirrhosis with rifampin. Results of a double-blind, crossover, randomized trial. Gastroenterology [Internet]. 1988 [cited 2023 Dec 15];94(2):488–493. Available from: https://pubmed.ncbi.nlm.nih.gov/3275568/
   PubMed Web of Science ®Google Scholar
• Podesta A, Lopez P, Terg R, et al. Treatment of pruritus of primary biliary cirrhosis with rifampin. Dig Dis Sci [Internet]. 1991 [cited 2023 Dec 15];36(2):216–220. Available from: https://pubmed.ncbi.nlm.nih.gov/1988266/
   PubMed Web of Science ®Google Scholar
• Bachs L, Parés A, Elena M, et al. Effects of long-term rifampicin administration in primary biliary cirrhosis. Gastroenterology [Internet]. 1992 [cited 2023 Dec 15];102(6):2077–2080. Available from: https://pubmed.ncbi.nlm.nih.gov/1587427/
   PubMed Web of Science ®Google Scholar
• Browning J, Combes B, Mayo MJ. Long-term efficacy of sertraline as a treatment for cholestatic pruritus in patients with primary biliary cirrhosis. Am J Gastroenterol [Internet]. 2003 [cited 2023 Dec15];98(12):2736–2741. Available from: https://pubmed.ncbi.nlm.nih.gov/14687826/
   PubMed Web of Science ®Google Scholar
• Mayo MJ, Handem I, Saldana S, et al. Sertraline as a first-line treatment for cholestatic pruritus. Hepatology [Internet]. 2007 [cited 2023 Dec 15];45(3):666–674. Available from: https://pubmed.ncbi.nlm.nih.gov/17326161/
   PubMed Web of Science ®Google Scholar
• Bergasa NV, Talbot TL, Alling DW, et al. A controlled trial of naloxone infusions for the pruritus of chronic cholestasis. Gastroenterology [Internet]. 1992 [cited 2023 Dec 15];102(2):544–549. Available from: https://pubmed.ncbi.nlm.nih.gov/1732125/
   PubMed Web of Science ®Google Scholar
• Bergasa NV, Ailing DW, Talbot TL, et al. Effects of naloxone infusions in patients with the pruritus of cholestasis. A double-blind, randomized, controlled trial. Ann Intern Med. 1995 [cited 2023 Dec 15];123(3):161–167. Available from: https://pubmed.ncbi.nlm.nih.gov/7598296/
   PubMed Web of Science ®Google Scholar
• Wolfhagen F, Sternieri E, Hop W, et al. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology. 1997;113(4):1264–1269. doi: 10.1053/gast.1997.v113.pm9322521
   PubMed Web of Science ®Google Scholar
• Terg R, Coronel E, Sordá J, et al. Efficacy and safety of oral naltrexone treatment for pruritus of cholestasis, a crossover, double blind, placebo-controlled study. J Hepatol. 2002;37(6):717–722. doi: 10.1016/S0168-8278(02)00318-5
   PubMed Web of Science ®Google Scholar
• Salic K, Kleemann R, Wilkins-Port C, et al. Apical sodium-dependent bile acid transporter inhibition with volixibat improves metabolic aspects and components of non-alcoholic steatohepatitis in Ldlr-/-.Leiden mice. PLOS ONE. 2019 [cited 2023 Dec 15];14(6):e0218459. Available from: /pmc/articles/PMC6590809/
   PubMed Web of Science ®Google Scholar
• Mirum Pharmaceuticals Inc. A study to evaluate efficacy and safety of an investigational drug named volixibat in patients with itching caused by primary biliary cholangitis (VANTAGE) [Internet]. [cited 2023 Dec 15]. Available from: https://clinicaltrials.gov/study/NCT05050136#study-plan
   Google Scholar
• Mirum Pharmaceuticals Inc. A study to evaluate efficacy and safety of an investigational drug named volixibat in patients with itching caused by primary sclerosing cholangitis (PSC) (VISTAS) [Internet]. [cited 2023 Dec 15]. Available from: https://clinicaltrials.gov/study/NCT04663308
   Google Scholar
• Thompson RJ, Arnell H, Artan R, et al. Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial. Lancet Gastroenterol Hepatol [Internet]. 2022 [cited 2023 Dec 15];7(9):830–842. Available from: http://www.thelancet.com/article/S2468125322000930/fulltext
   PubMed Web of Science ®Google Scholar
• Baghdasaryan A, Fuchs CD, Österreicher CH, et al. Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis. J Hepatol [Internet]. 2016 [cited 2023 Dec 15];64(3):674–681. Available from: http://www.journal-of-hepatology.eu/article/S0168827815007230/fulltext
   PubMed Web of Science ®Google Scholar
• Al-Dury S, Wahlström A, Wahlin S, et al. Pilot study with IBAT inhibitor A4250 for the treatment of cholestatic pruritus in primary biliary cholangitis. Sci Rep. 2018 [cited 2023 Dec 15];8(1). Available from: /pmc/articles/PMC5923243/
   PubMedGoogle Scholar
• Deeks ED. Odevixibat: First Approval. Drugs [Internet]. 2021 [cited 2023 Dec 15];81(15):1781. Available from: /pmc/articles/PMC8550539/
   Google Scholar
• Mayo MJ, Pockros PJ, Jones D, et al. A randomized, controlled, phase 2 study of maralixibat in the treatment of itching associated with primary biliary cholangitis. Hepatol Commun [Internet]. 2019 [cited 2023 Dec 15];3(3):365–381. Available from: https://pubmed.ncbi.nlm.nih.gov/30859149/
   PubMed Web of Science ®Google Scholar
• Gonzales E, Hardikar W, Stormon M, et al. Efficacy and safety of maralixibat treatment in patients with alagille syndrome and cholestatic pruritus (ICONIC): a randomised phase 2 study. Lancet. 2021 [cited 2023 Dec 15];398(10311):1581–1592. Available from: http://www.thelancet.com/article/S0140673621012563/fulltext
   PubMed Web of Science ®Google Scholar
• Shirley M. Maralixibat: First Approval. Drugs [Internet]. 2022 [cited 2023 Dec 15];82(1):71. Available from: /pmc/articles/PMC8748361/
   Google Scholar
• Levy C, Kendrick S, Bowlus CL, et al. GLIMMER: a randomized phase 2b dose-ranging trial of linerixibat in primary biliary cholangitis patients with pruritus. Clin Gastroenterol Hepatol [Internet]. 2023 [cited 2023 Dec 15];21(7):1902–1912.e13. Available from: https://pubmed.ncbi.nlm.nih.gov/36343847/
   PubMed Web of Science ®Google Scholar
• Hegade VS, Kendrick SFW, Dobbins RL, et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. Lancet. 2017 [cited 2023 Dec 15];389(10074):1114–1123. Available from: http://www.thelancet.com/article/S0140673617303197/fulltext
   PubMed Web of Science ®Google Scholar
• Kirby J, Burton JL, Heaton KW. Pruritic effect of bile salts. Br Med J [Internet]. 1974 [cited 2023 Dec 15];4(5946):693. Available from /pmc/articles/PMC1612871/?report=abstract
   PubMed Web of Science ®Google Scholar
• Varadi DP. Pruritus induced by crude bile and purified bile acids: experimental production of pruritus in human skin. Arch Dermatol [Internet]. 1974 [cited 2023 Dec 15];109(5):678–681. Available from: https://jamanetwork.com/journals/jamadermatology/fullarticle/533983
   PubMedGoogle Scholar
• Alemi F, Kwon E, Poole DP, et al. The TGR5 receptor mediates bile acid–induced itch and analgesia. J Clin Invest. 2013 [cited 2023 Dec 15];123(4):1513. Available from: /pmc/articles/PMC3613908/
   PubMed Web of Science ®Google Scholar
• Lieu T, Jayaweera G, Zhao P, et al. The bile acid receptor TGR5 activates the TRPA1 channel to induce itch in mice. Gastroenterology [Internet]. 2014 [cited 2023 Dec 15];147:1417. Available from: /pmc/articles/PMC4821165/
   PubMed Web of Science ®Google Scholar
• Cipriani S, Renga B, D’Amore C, et al. Impaired itching perception in murine models of cholestasis is supported by dysregulation of GPBAR1 signaling. PLOS ONE. 2015 [cited 2023 Dec 15];10(7):e0129866. Available from: /pmc/articles/PMC4503431/
   PubMed Web of Science ®Google Scholar
• Murphy GM, Ross A, Billing BH. Serum bile acids in primary biliary cirrhosis. Gut [Internet]. 1972 [cited 2023 Dec 15];13(3):201–206. Available from: https://pubmed.ncbi.nlm.nih.gov/5024725/
   Google Scholar
• Ghent CN, Bloomer JR, Klatskin G. Elevations in skin tissue levels of bile acids in human cholestasis: relation to serum levels and topruritus. Gastroenterology. 1977;73(5):1125–1130. doi: 10.1016/S0016-5085(19)31870-0
   PubMed Web of Science ®Google Scholar
• Ghent CN, Bloomer JR. Itch in liver disease: facts and speculations. Yale J Biol Med [Internet]. 1979 [cited 2023 Dec 15];52:77. Available from: /pmc/articles/PMC2595714/?report=abstract
   PubMed Web of Science ®Google Scholar
• Bartholomew TC, Summerfield JA, Billing BH, et al. Bile acid profiles of human serum and skin interstitial fluid and their relationship to pruritus studied by gas chromatography–mass spectrometry. Clin Sci. 1982 [cited 2023 Dec 15];63(1):65–73. Available from: /clinsci/article/63/1/65/72927/Bile-Acid-Profiles-of-Human-Serum-and-Skin
   Web of Science ®Google Scholar
• Hirschfield GM, Dyson JK, Alexander GJM, et al. The british society of gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut BMJ Pub Group. 2018;67(9):1568–1594.
   PubMed Web of Science ®Google Scholar
• Oster ZH, Rachmilewitz EA, Moran E, et al. Relief of pruritus by cholestyramine in chronic liver disease. Isr J Med Sci. 1965;1(4):599–606.
   PubMedGoogle Scholar
• Datta DV, Sherlock S. Treatment Of pruritus of obstructive jaundice with cholestyramine. Br Med J [Internet]. 1963 [cited 2023 Dec 15];1(5325):216. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2123703/
   PubMedGoogle Scholar
• Datta DV, Sherlock S. Cholestyramine for long term relief of the pruritus complicating intrahepatic cholestasis. Gastroenterology. 1966;50(3):323–332. doi: 10.1016/S0016-5085(66)80071-9
   PubMed Web of Science ®Google Scholar
• Duncan JS, Kennedy HJ, Triger DR. Treatment of pruritus due to chronic obstructive liver disease. Br Med J (Clin Res Ed) [Internet]. 1984 [cited 2023 Dec 15];289(6436):22. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1442057/
   PubMedGoogle Scholar
• Di Padova C, Tritapepe R, Rovagnati P, et al. Double-blind placebo-controlled clinical trial of microporous cholestyramine in the treatment of intra- and extra-hepatic cholestasis: relationship between itching and serum bile acids. Methods Find Exp Clin Pharmacol. 1984;6(12):773–776.
   PubMedGoogle Scholar
• Kuiper EMM, van Erpecum KJ, Beuers U, et al. The potent bile acid sequestrant colesevelam is not effective in cholestatic pruritus: results of a double-blind, randomized, placebo-controlled trial. Hepatology. 2010;52(4):1334–1340. doi: 10.1002/hep.23821
   PubMed Web of Science ®Google Scholar
• Hegade VS, Krawczyk M, Kremer AE, et al. The safety and efficacy of nasobiliary drainage in the treatment of refractory cholestatic pruritus: a multicentre European study. Aliment Pharmacol Ther [Internet]. 2016 [cited 2023 Dec 15];43(2):294–302. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/apt.13449
   PubMed Web of Science ®Google Scholar
• Jankowska I, Czubkowski P, Kaliciński P, et al. Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr. 2014 [cited 2023 Dec 15];58(1):92–95. Available from: https://journals.lww.com/jpgn/fulltext/2014/01000/ileal_exclusion_in_children_with_progressive.23.aspx
   PubMed Web of Science ®Google Scholar
• Ramachandran P, Shanmugam NP, Al SS, et al. Outcome of partial internal biliary diversion for intractable pruritus in children with cholestatic liver disease. Pediatr Surg Int [Internet]. 2014 [cited 2023 Dec 15];30(10):1045–1049. Available from: https://link.springer.com/article/10.1007/s00383-014-3559-x
   PubMed Web of Science ®Google Scholar
• Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology. 1988;95(1):130–136. doi: 10.1016/0016-5085(88)90301-0
   PubMed Web of Science ®Google Scholar
• Schaefer B, Schaefer F, Wittmer D, et al. Molecular adsorbents recirculating system dialysis in children with cholestatic pruritus. Pediatr Nephrol. 2012 [cited 2023 Dec 15];27(5):829–834. Available from: https://link.springer.com/article/10.1007/s00467-011-2058-8
   PubMed Web of Science ®Google Scholar
• Krawczyk M, Liebe R, Wasilewicz M, et al. Plasmapheresis exerts a long-lasting antipruritic effect in severe cholestatic itch. Liver International [Internet]. 2017 [cited 2023 Dec 15];37(5):743–747. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/liv.13281
   PubMed Web of Science ®Google Scholar
• Kittanamongkolchai W, El-Zoghby ZM, Eileen Hay J, et al. Charcoal hemoperfusion in the treatment of medically refractory pruritus in cholestatic liver disease. Hepatol Int [Internet]. 2017 [cited 2023 Dec 15];11(4):384–389. Available from: https://link.springer.com/article/10.1007/s12072-016-9775-9
   PubMed Web of Science ®Google Scholar
• Decock S, Roelandts R, Van SW, et al. Cholestasis-induced pruritus treated with ultraviolet B phototherapy: an observational case series study. J Hepatol [Internet]. 2012 [cited 2023 Dec 15];57(3):637–641. Available from: http://www.journal-of-hepatology.eu/article/S0168827812003455/fulltext
   PubMed Web of Science ®Google Scholar
• Takahashi N, Tominaga M, Kosaka R, et al. Involvement of µ-opioid receptors and κ-opioid receptors in itch-related scratching behaviour of imiquimod-induced psoriasis-like dermatitis in mice. Acta Derm Venerol. 2017 [cited 2023 Dec 15];97(8):928–933. Available from: https://medicaljournalssweden.se/actadv/article/view/5155
   PubMed Web of Science ®Google Scholar
• Hales P. Pruritus after epidural morphine. Lancet. 1980;316(8187):204. doi: 10.1016/S0140-6736(80)90090-2
   Google Scholar
• Duffy BL. Itching as a side-effect of epidural morphine. Anaesthesia. 1981;36(1):67. doi: 10.1111/j.1365-2044.1981.tb08609.x
   PubMed Web of Science ®Google Scholar
• Ballantyne JC, Loach AB, Carr DB. Itching after epidural and spinal opiates. Pain. 1988 [cited 2023 Dec 15];33(2):149–160. Available from: https://journals.lww.com/pain/fulltext/1988/05000/itching_after_epidural_and_spinal_opiates.2.aspx
   PubMed Web of Science ®Google Scholar
• Benson JL, Campbell HE, Phillips CN. Opioid-induced pruritus. Consult Pharm. 2015;30(4):221–227. doi: 10.4140/TCP.n.2015.221
   PubMedGoogle Scholar
• Liu XY, Liu ZC, Sun YG, et al. Unidirectional cross-activation of GRPR by MOR1D uncouples itch and analgesia induced by opioids. Cell. 2011 [cited 2023 Dec 15];147(2):447. Available from: /pmc/articles/PMC3197217/
   PubMed Web of Science ®Google Scholar
• Thornton JR, Losowsky MS. Plasma methionine enkephalin concentration and prognosis in primary biliary cirrhosis. BMJ-BRIT MED J [Internet]. 1988 [cited 2023 Dec 15];297(6658):1241. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1834726/
   PubMedGoogle Scholar
• Spivey JR, Jorgensen RA, Gores GJ, et al. Methionine-enkephalin concentrations correlate with stage of disease but not pruritus in patients with primary biliary cirrhosis. Am J Gastroenterol. 1994;89(11):2028–2032.
   PubMed Web of Science ®Google Scholar
• Swain MG, Rothman RB, Xu H, et al. Endogenous opioids accumulate in plasma in a rat model of acute cholestasis. Gastroenterology. 1992;103(2):630–635. doi: 10.1016/0016-5085(92)90857-U
   PubMed Web of Science ®Google Scholar
• Bergasa NV, Rothman RB, Vergalla J, et al. Central mu-opioid receptors are down-regulated in a rat model of cholestasis. J Hepatol [Internet]. 1992 [cited 2023 Dec 15];15(1–2):220–224. Available from: http://www.journal-of-hepatology.eu/article/016882789290040V/fulltext
   PubMed Web of Science ®Google Scholar
• Thornton JR, Losowsky MS. Opioid peptides and primary biliary cirrhosis. BMJ. 1988;297(6662):1501–1504. doi: 10.1136/bmj.297.6662.1501
   PubMedGoogle Scholar
• Jones EA, Dekker LR. Florid opioid withdrawal–like reaction precipitated by naltrexone in a patient with chronic cholestasis. Gastroenterology. 2000;118(2):431–432. doi: 10.1016/S0016-5085(00)70225-3
   PubMed Web of Science ®Google Scholar
• Mansour-Ghanaei F, Taheri A, Froutan H, et al. Effect of oral naltrexone on pruritus in cholestatic patients. World J Gastroenterol: WJG [Internet]. 2006 [cited 2023 Dec 15];12(7):1125. Available from: /pmc/articles/PMC4087908/
   PubMed Web of Science ®Google Scholar
• Shawcross DL, Jalan R. Delayed opioid withdrawal-like reaction in primary biliary cirrhosis following naloxone therapy. Gastroenterology [Internet]. 2001 [cited 2023 Dec 15];121(3):743–744. Available from https://pubmed.ncbi.nlm.nih.gov/11547785/
   PubMed Web of Science ®Google Scholar
• Moolenaar WH, Perrakis A. Insights into autotaxin: how to produce and present a lipid mediator. Nat Rev Mol Cell Biol [Internet]. 2011 [cited 2023 Dec15];12(10):674–679. Available from: https://pubmed.ncbi.nlm.nih.gov/21915140/
   PubMed Web of Science ®Google Scholar
• Hashimoto T, Ohata H, Momose K. Itch-scratch responses induced by lysophosphatidic acid in mice. Pharmacology [Internet]. 2004;72(1):51–56. [cited 2023 Dec 15]. Available from: https://pubmed.ncbi.nlm.nih.gov/15292655/
   Google Scholar
• Kittaka H, Uchida K, Fukuta N, et al. Lysophosphatidic acid-induced itch is mediated by signalling of LPA5 receptor, phospholipase D and TRPA1/TRPV1. J Physiol. 2017 [cited 2023 Dec 15];595(8):2681–2698. Available from: https://pubmed.ncbi.nlm.nih.gov/28176353/
   PubMed Web of Science ®Google Scholar
• Sun Y, Zhang W, Evans JF, et al. Autotaxin, pruritus and primary biliary cholangitis (PBC). Autoimmun Rev [Internet]. 2016 [cited 2023 Dec 15];15(8):795–800. Available from: https://pubmed.ncbi.nlm.nih.gov/27019050/
   PubMed Web of Science ®Google Scholar
• Kremer AE, van Dijk R, Leckie P, et al. Serum autotaxin is increased in pruritus of cholestasis, but not of other origin, and responds to therapeutic interventions. Hepatology [Internet]. 2012 [cited 2023 Dec 15];56(4):1391–1400. Available from: https://pubmed.ncbi.nlm.nih.gov/22473838/
   PubMed Web of Science ®Google Scholar
• Hegade VS, Pechlivanis A, McDonald JAK, et al. Autotaxin, bile acid profile and effect of ileal bile acid transporter inhibition in primary biliary cholangitis patients with pruritus. Liver Int [Internet]. 2019 [cited 2023 Dec 15];39(5):967–975. Available from: https://pubmed.ncbi.nlm.nih.gov/30735608/
   PubMed Web of Science ®Google Scholar
• Joshita S, Umemura T, Usami Y, et al. Serum autotaxin is a useful disease progression marker in patients with primary biliary cholangitis. Sci Rep. 2018 [cited 2023 Dec 15];8(1). Available from: https://pubmed.ncbi.nlm.nih.gov/29802350/
   Google Scholar
• Wunsch E, Krawczyk M, Milkiewicz M, et al. Serum autotaxin is a marker of the severity of liver injury and overall survival in patients with cholestatic liver diseases. Sci Rep. 2016 [cited 2023 Dec 15];6(1). Available from: /pmc/articles/PMC4978954/
   Google Scholar
• Nakao M, Sugaya M, Suga H, et al. Serum autotaxin levels correlate with pruritus in patients with atopic dermatitis. J Invest Dermatol. 2014 [cited 2023 Dec 15];134(6):1745–1747. Available from: https://pubmed.ncbi.nlm.nih.gov/24441098/
   PubMed Web of Science ®Google Scholar
• Joshita S, Ichikawa Y, Umemura T, et al. Serum autotaxin is a useful liver fibrosis marker in patients with chronic hepatitis B virus infection. Hepatol Res [Internet]. 2018 [cited 2023 Dec 15];48(4):275–285. Available from: https://pubmed.ncbi.nlm.nih.gov/29114991/
   Web of Science ®Google Scholar
• Yamazaki T, Joshita S, Umemura T, et al. Association of serum autotaxin levels with liver fibrosis in patients with chronic hepatitis C. Sci Rep. 2017 [cited 2023 Dec 15];7(1). Available from: https://pubmed.ncbi.nlm.nih.gov/28425454/
   Google Scholar
• Fujimori N, Umemura T, Kimura T, et al. Serum autotaxin levels are correlated with hepatic fibrosis and ballooning in patients with non-alcoholic fatty liver disease. World J Gastroenterol [Internet]. 2018 [cited 2023 Dec 15];24(11):1239–1249. Available from: https://pubmed.ncbi.nlm.nih.gov/29568204/
   PubMed Web of Science ®Google Scholar
• Patel SP, Vasavda C, Ho B, et al. Cholestatic pruritus: emerging mechanisms and therapeutics. J Am Acad Dermatol [Internet]. 2019 [cited 2023 Dec 15];81(6):1371–1378. Available from: https://pubmed.ncbi.nlm.nih.gov/31009666/
   PubMed Web of Science ®Google Scholar
• Tolson AH, Wang H. Regulation of drug-metabolizing enzymes by xenobiotic receptors: PXR and CAR. Adv Drug Deliv Rev [Internet]. 2010 [cited 2023 Dec 15];62(13):1238–1249. Available from: https://pubmed.ncbi.nlm.nih.gov/20727377/
   PubMed Web of Science ®Google Scholar
• Cynamon HA, Andres JM, Iafrate RP. Rifampin relieves pruritus in children with cholestatic liver disease. Gastroenterology [Internet]. 1990 [cited 2023 Dec 15];98(4):1013–1016. Available from: https://pubmed.ncbi.nlm.nih.gov/2179027/
   PubMed Web of Science ®Google Scholar
• Webb GJ, Rahman SR, Levy C, et al. Low risk of hepatotoxicity from rifampicin when used for cholestatic pruritus: a cross-disease cohort study. Aliment Pharmacol Ther [Internet]. 2018 [cited 2023 Dec 15];47(8):1213–1219. Available from: https://pubmed.ncbi.nlm.nih.gov/29468705/
   PubMed Web of Science ®Google Scholar
• Covic A, Goldsmith DJA, Segall L, et al. Rifampicin-induced acute renal failure: a series of 60 patients. Nephrol Dial Transplant [Internet]. 1998 [cited 2023 Dec 15];13(4):924–929. Available from: https://pubmed.ncbi.nlm.nih.gov/9568851/
   PubMed Web of Science ®Google Scholar
• Tian B, Wang XL, Huang Y, et al. Peripheral and spinal 5-HT receptors participate in cholestatic itch and antinociception induced by bile duct ligation in rats. Sci Rep. 2016 [cited 2023 Dec 15];6(1). Available from: https://pubmed.ncbi.nlm.nih.gov/27824106/
   Google Scholar
• Morita T, McClain SP, Batia LM, et al. HTR7 mediates serotonergic acute and chronic itch. Neuron [Internet]. 2015 [cited 2023 Dec 15];87(1):124–138. Available from: https://pubmed.ncbi.nlm.nih.gov/26074006/
   PubMed Web of Science ®Google Scholar
• Gupta MA, Gupta AK, Schork NJ, et al. Depression modulates pruritus perception: a study of pruritus in psoriasis, atopic dermatitis, and chronic idiopathic urticaria. Psychosom Med [Internet]. 1994 [cited 2023 Dec 15];56(1):36–40. Available from: https://pubmed.ncbi.nlm.nih.gov/8197313/
   PubMed Web of Science ®Google Scholar
• Ataei S, Kord L, Larki A, et al. Comparison of sertraline with rifampin in the treatment of cholestatic pruritus: a randomized clinical trial. Rev Recent Clin Trials [Internet]. 2019 [cited 2023 Dec 15];14(3):217–223. Available from: https://pubmed.ncbi.nlm.nih.gov/30919782/
   PubMed Web of Science ®Google Scholar
• Reig A, Sesé P, Parés A. Effects of bezafibrate on outcome and pruritus in primary biliary cholangitis with suboptimal ursodeoxycholic acid response. Am J Gastroenterol [Internet]. 2018 [cited 2023 Dec15];113(1):49–55. Available from: https://pubmed.ncbi.nlm.nih.gov/29016567/
   PubMed Web of Science ®Google Scholar
• Boyer JL. Bile formation and secretion. Compr Physiol [Internet]. 2013 [cited 2023 Dec 15];3:1035–1078. Available from: https://pubmed.ncbi.nlm.nih.gov/23897680/
   PubMed Web of Science ®Google Scholar
• Staels B, Fonseca VA. Bile acids and metabolic regulation: mechanisms and clinical responses to bile acid sequestration. Diabetes Care [Internet]. 2009 [cited 2023 Dec 15];32(suppl_2):S237. Available from: /pmc/articles/PMC2811459/
   PubMedGoogle Scholar
• Dawson PA. Role of the intestinal bile acid transporters in bile acid and drug disposition. Handb Exp Pharmacol [Internet]. 2011 [cited 2023 Dec 15];201:169–203. Available from: https://pubmed.ncbi.nlm.nih.gov/21103970/
   Google Scholar
• Slavetinsky C, Sturm E. Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis. BMJ Case Rep. 2020;13(6):e234185. doi: 10.1136/bcr-2019-234185
   PubMedGoogle Scholar
• Miethke AG, Zhang W, Simmons J, et al. Pharmacological inhibition of apical sodium-dependent bile acid transporter changes bile composition and blocks progression of sclerosing cholangitis in multidrug resistance 2 knockout mice. Hepatology [Internet]. 2016 [cited 2023 Dec 15];63(2):512–523. Available from: https://pubmed.ncbi.nlm.nih.gov/26172874/
   PubMed Web of Science ®Google Scholar
• Truong JK, Bennett AL, Klindt C, et al. Ileal bile acid transporter inhibition in Cyp2c70 KO mice ameliorates cholestatic liver injury. J Lipid Res [Internet]. 2022 [cited 2023 Dec 15];63(9):100261. Available from: https://pubmed.ncbi.nlm.nih.gov/35934110/
   PubMed Web of Science ®Google Scholar
• Yamauchi R, Takedatsu H, Yokoyama K, et al. Elobixibat, an ileal bile acid transporter inhibitor, ameliorates non-alcoholic steatohepatitis in mice. Hepatol Int [Internet]. 2021 [cited 2023 Dec 15];15(2):392–404. Available from: https://link.springer.com/article/10.1007/s12072-020-10107-0
   PubMed Web of Science ®Google Scholar
• Rao A, Kosters A, Mells JE, et al. Inhibition of ileal bile acid uptake protects against nonalcoholic fatty liver disease in high-fat diet–fed mice. Sci Transl Med. 2016 [cited 2023 Dec 15];8(357):357ra122. Available from: /pmc/articles/PMC5056562/
   PubMed Web of Science ®Google Scholar
• Ino H, Endo A, Wakamatsu A, et al. Safety, tolerability, pharmacokinetic and pharmacodynamic evaluations following single oral doses of GSK2330672 in healthy Japanese volunteers. Clin Pharmacol Drug Dev [Internet]. 2019 [cited 2023 Dec 15];8(1):70–77. Available from: https://onlinelibrary.wiley.com/doi/full/10.1002/cpdd.576
   PubMed Web of Science ®Google Scholar
• Zamek-Gliszczynski MJ, Kenworthy D, Bershas DA, et al. Pharmacokinetics and ADME characterization of intravenous and oral [14C]-linerixibat in healthy male volunteers. Drug Metab Dispos. 2021 [cited 2023 Dec 15];49(12):1109–1117. Available from: https://dmd.aspetjournals.org/content/49/12/1109
   PubMedGoogle Scholar
• Siebers N, Palmer M, Silberg DG, et al. Absorption, distribution, metabolism, and excretion of [14C]-volixibat in healthy men: phase 1 open-label study. Eur J Drug Metab Pharmacokinet [Internet]. 2018 [cited 2023 Dec 15];43(1):91. Available from: /pmc/articles/PMC5794849/
   PubMed Web of Science ®Google Scholar
• Mirum pharmaceuticals international B.V. Annex i summary of product characteristics [Internet]. 2022 [cited 2023 Dec 15]. Available from: https://www.ema.europa.eu/en/documents/product-information/livmarli-epar-product-information_en.pdf.
   Google Scholar
• Albireo AB Annex i summary of product characteristics [Internet]. 2021 [cited 2023 Dec 15]. Available from: https://www.ema.europa.eu/en/documents/product-information/bylvay-epar-product-information_en.pdf
   Google Scholar
• Graffner H, Gillberg PG, Rikner L, et al. The ileal bile acid transporter inhibitor A4250 decreases serum bile acids by interrupting the enterohepatic circulation. Aliment Pharmacol Ther [Internet]. 2016 [cited 2023 Dec 15];43(2):303–310. Available from: https://onlinelibrary.wiley.com/doi/full/10.1111/apt.13457
   PubMed Web of Science ®Google Scholar
• Palmer M, Jennings L, Silberg DG, et al. A randomised, double-blind, placebo-controlled phase 1 study of the safety, tolerability and pharmacodynamics of volixibat in overweight and obese but otherwise healthy adults: implications for treatment of non-alcoholic steatohepatitis. BMC Pharmacol Toxicol [Internet]. 2018 [cited 2023 Dec 15];19(1). Available from: /pmc/articles/PMC5857122/
   Google Scholar
• Tiessen RG, Kennedy CA, Keller BT, et al. Safety, tolerability and pharmacodynamics of apical sodium-dependent bile acid transporter inhibition with volixibat in healthy adults and patients with type 2 diabetes mellitus: a randomised placebo-controlled trial. BMC Gastroenterol [Internet]. 2018 [cited 2023 Dec 15];18(1):3. Available from: /pmc/articles/PMC5756385/
   PubMedGoogle Scholar
• Kunst RF, de Waart DR, Wolters F, et al. Systemic ASBT inactivation protects against liver damage in obstructive cholestasis in mice. JHEP Reports [Internet]. 2022 [cited 2023 Dec 15];4(11):100573. Available from: /pmc/articles/PMC9494276/
   PubMedGoogle Scholar
• Caballero-Camino FJ, Rodrigues PM, Wångsell F, et al. A3907, a systemic ASBT inhibitor, improves cholestasis in mice by multiorgan activity and shows translational relevance to humans. Hepatology [Internet]. 2023 [cited 2023 Dec 15];78(3):709. Available from: /pmc/articles/PMC10442107/
   PubMed Web of Science ®Google Scholar
• Newsome PN, Palmer M, Freilich B, et al. Volixibat in adults with non-alcoholic steatohepatitis: 24-week interim analysis from a randomized, phase II study. J Hepatol [Internet]. 2020 [cited 2023 Dec 15];73(2):231–240. Available from: http://www.journal-of-hepatology.eu/article/S0168827820301860/fulltext
   PubMed Web of Science ®Google Scholar
• GlaxoSmithKline. Global linerixibat itch study of efficacy and safety in primary biliary cholangitis (PBC) (GLISTEN) [Internet]. [cited 2023 Dec 15]. Available from: https://clinicaltrials.gov/study/NCT04950127#study-plan
   Google Scholar
• NICE. Odevixibat for treating progressive familial intrahepatic cholestasis [Internet]. 2022 [cited 2023 Dec 15]. Available from: https://www.nice.org.uk/guidance/HST17
   Google Scholar
• European Medicines Agency. Livmarli [Internet]. 2023 [cited 2023 Dec 15]. Available from: https://www.ema.europa.eu/en/medicines/human/EPAR/livmarli
   Google Scholar