[1] Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut, 2018,67(9):1568-1594. [2] Younossi ZM, Bernstein D, Shiffman ML, et al. Diagnosis and management of primary biliary cholangitis. Am J Gastroenterol, 2019, 114(1):48-63. [3] 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(1):145-172. [4] Al-Harthy N, Kumagi T. Natural history and management of primary biliary cirrhosis. Hepat Med, 2012, 4:61-71. [5] Shah RA, Kowdley KV. Current and potential treatments for primary biliary cholangitis. Lancet Gastroenterol Hepatol, 2020,5(3):306-315. [6] 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(1):394-419. [7] Poupon R, Chrétien Y, Poupon RE, et al. Is ursodeoxycholic acid an effective treatment for primary biliary cirrhosis? Lancet, 1987, 1(8537):834-836. [8] Harms MH, van Buuren HR, Corpechot C, et al. Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. J Hepatol, 2019, 71(2):357-365. [9] Harms MH, de Veer RC, Lammers WJ, et al. Number needed to treat with ursodeoxycholic acid therapy to prevent liver transplantation or death in primary biliary cholangitis. Gut, 2020, 69(8):1502-1509. [10] You H, Ma X, Efe C, et al. APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis. Hepatol Int, 2022, 16(1):1-23. [11] Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med, 2016, 375(7):631-643. [12] Samur S, Klebanoff M, Banken R, et al. Long-term clinical impact and cost-effectiveness of obeticholic acid for the treatment of primary biliary cholangitis. Hepatology, 2017, 65(3):920-928. [13] Kowdley KV, Luketic V, Chapman R, et al. A randomized trial of obeticholic acid monotherapy in patients with primary biliary cholangitis. Hepatology, 2018, 67(5):1890-1902. [14] Trauner M, Nevens F, Shiffman ML, et al. Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study. Lancet Gastroenterol Hepatol, 2019, 4(6):445-453. [15] Lleo A, Wang GQ, Gershwin ME, et al. Primary biliary cholangitis. Lancet, 2020,396(10266):1915-1926. [16] Ghonem NS, Assis DN, Boyer JL. Fibrates and cholestasis. Hepatology, 2015, 62(2):635-643. [17] Jones D, Boudes PF, Swain MG, et al. Seladelpar (MBX-8025), a selective PPAR-δ agonist, in patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid: a double-blind, randomised, placebo-controlled, phase 2, proof-of-concept study. Lancet Gastroenterol Hepatol, 2017, 2(10):716-726. [18] Chascsa DMH, Lindor KD. Emerging therapies for PBC. J Gastroenterol, 2020, 55(3):261-272. [19] Cheung AC, Lapointe-Shaw L, Kowgier M, et al. Combined ursodeoxycholic acid (UDCA) and fenofibrate in primary biliary cholangitis patients with incomplete UDCA response may improve outcomes. Aliment Pharmacol Ther, 2016, 43(2):283-293. [20] Corpechot C, Chazouillères O, Rousseau A, et al. A placebo-controlled trial of bezafibrate in primary biliary cholangitis. N Engl J Med, 2018, 378(23):2171-2181. [21] Reig A, Sesé P, Parés A.et al.Effects of bezafibrate on outcome and pruritus in primary biliary cholangitis with suboptimal ursodeoxycholic acid response. Am J Gastroenterol, 2018, 113(1):49-55. [22] Sorda JA, González Ballerga E, Barreyro FJ, et al. Bezafibrate therapy in primary biliary cholangitis refractory to ursodeoxycholic acid: a longitudinal study of paired liver biopsies at 5?years of follow up. Aliment Pharmacol Ther, 2021, 54(9):1202-1212. [23] Chung SW, Lee JH, Kim MA, et al. Additional fibrate treatment in UDCA-refractory PBC patients. Liver Int, 2019, 39(9):1776-1785. [24] 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, 2021, 74(6):1344-1354. [25] Vuppalanchi R, Caldwell SH, Pyrsopoulos N, et al. Proof-of-concept study to evaluate the safety and efficacy of saroglitazar in patients with primary biliary cholangitis. J Hepatol, 2022, 76(1):75-85. [26] Chascsa DMH, Lindor KD. Emerging therapies for PBC. J Gastroenterol. 2020 ,55(3):261-272. [27] Leung J, Bonis PA, Kaplan MM. Colchicine or methotrexate, with ursodiol, are effective after 20 years in a subset of patients with primary biliary cirrhosis. Clin Gastroenterol Hepatol, 2011, 9(9):776-780. [28] Rautiainen H, Kärkkäinen P, Karvonen AL, et al. Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial. Hepatology, 2005, 41(4):747-752. [29] Hirschfield GM, Beuers U, Kupcinskas L,et al. A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA. J Hepatol, 2021,74(2):321-329. [30] Myers RP, Swain MG, Lee SS, et al. B-cell depletion with rituximab in patients with primary biliary cirrhosis refractory to ursodeoxycholic acid. Am J Gastroenterol, 2013,108(6):933-941. [31] Khanna A, Jopson L, Howel D, et al. Rituximab is ineffective for treatment of fatigue in primary biliary cholangitis: A phase 2 randomized controlled trial. Hepatology, 2019,70(5):1646-1657. [32] Eckmann JD, Chedid V, Quinn KP, et al. De novo colitis associated with rituximab in 21 patients at a tertiary center. Clin Gastroenterol Hepatol, 2020,18(1):252-253. [33] Tanaka Y, Takeuchi T, Yamanaka H,et al. Efficacy and safety of e6011, an anti-fractalkine monoclonal antibody, in patients with active rheumatoid arthritis with inadequate response to methotrexate: results of a randomized, double-blind, placebo-controlled phase Ⅱ study. Arthritis Rheumatol, 2021,3(4):587-595. [34] Tang R, Wei Y, Li Y, et al. Gut microbial profile is altered in primary biliary cholangitis and partially restored after UDCA therapy. Gut, 2018, 67(3):534-541. [35] Giordano DM, Pinto C, Maroni L, et al. Inflammation and the gut-liver axis in the pathophysiology of cholangiopathies. Int J Mol Sci, 2018, 19(10):3003. [36] Wunsch E, Raszeja-Wyszomirska J, Barbier O, et al. Effect of S-adenosyl-L-methionine on liver biochemistry and quality of life in patients with primary biliary cholangitis treated with ursodeoxycholic acid. A prospective, open label pilot study. J Gastrointestin Liver Dis, 2018, 27(3):273-279. [37] Tong GD, Tang HH, Wei CS, et al. Efficacy of ursodeoxycholic acid combined with Tongdan Decoction (sic) on immunological indices and histopathological changes in primary biliary cirrhosis patients. Chin J Integr Med, 2012,18(1):16-22. |