Guidelines on the diagnosis and management of primary biliary cholangitis (2021)

Chinese Society of Hepatology

doi:10.3760/cma.j.cn112138-20211112-00794

Volume 38 Issue 1

Jan. 2022

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Article Navigation > Journal of Clinical Hepatology > 2022 > 38(1): 35-41

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Guidelines on the diagnosis and management of primary biliary cholangitis (2021)

DOI: 10.3760/cma.j.cn112138-20211112-00794

Chinese Society of Hepatology

Received Date: 2021-12-25
Published Date: 2022-01-20

Abstract

Abstract

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued the consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts assessed the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.
- Primary Biliary Cholangitis,
- Primary Biliary Cirrhosis,
- Diagnosis,
- Therapeutics,
- Practice Guideline

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References(113)

References

[1]	LV T, CHEN S, LI M, et al. Regional variation and temporal trend of primary biliary cholangitis epidemiology: A systematic review and meta-analysis[J]. J Gastroenterol Hepatol, 2021, 36(6): 1423-1434. DOI: 10.1111/jgh.15329.
[2]	ZENG N, DUAN W, CHEN S, et al. Epidemiology and clinical course of primary biliary cholangitis in the Asia-Pacific region: A systematic review and meta-analysis[J]. Hepatol Int, 2019, 13(6): 788-799. DOI: 10.1007/s12072-019-09984-x.
[3]	MAYO MJ. Natural history of primary biliary cirrhosis[J]. Clin Liver Dis, 2008, 12(2): 277-288; viii. DOI: 10.1016/j.cld.2008.02.012.
[4]	PARÉS A, CABALLERÍA L, RODÉS J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic Acid[J]. Gastroenterology, 2006, 130(3): 715-720. DOI: 10.1053/j.gastro.2005.12.029.
[5]	HARMS MH, van BUUREN HR, CORPECHOT C, et al. Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis[J]. J Hepatol, 2019, 71(2): 357-365. DOI: 10.1016/j.jhep.2019.04.001.
[6]	GRANITO A, MURATORI P, QUARNETI C, et al. Antinuclear antibodies as ancillary markers in primary biliary cirrhosis[J]. Expert Rev Mol Diagn, 2012, 12(1): 65-74. DOI: 10.1586/erm.11.82.
[7]	LEUNG PS, ROSSARO L, DAVIS PA, et al. Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis[J]. Hepatology, 2007, 46(5): 1436-1442. DOI: 10.1002/hep.21828.
[8]	ZHANG Q, LIU Z, WU S, et al. Meta-analysis of antinuclear antibodies in the diagnosis of antimitochondrial antibody-negative primary biliary cholangitis[J]. Gastroenterol Res Pract, 2019, 2019: 8959103. DOI: 10.1155/2019/8959103.
[9]	GRANITO A, MURATORI P, MURATORI L, et al. Antinuclear antibodies giving the 'multiple nuclear dots' or the 'rim-like/membranous' patterns: Diagnostic accuracy for primary biliary cirrhosis[J]. Aliment Pharmacol Ther, 2006, 24(11-12): 1575-1583. DOI: 10.1111/j.1365-2036.2006.03172.x.
[10]	CORPECHOT C, CARRAT F, POUJOL-ROBERT A, et al. Noninvasive elastography-based assessment of liver fibrosis progression and prognosis in primary biliary cirrhosis[J]. Hepatology, 2012, 56(1): 198-208. DOI: 10.1002/hep.25599.
[11]	LUDWIG J, DICKSON ER, MCDONALD GS. Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis)[J]. Virchows Arch A Pathol Anat Histol, 1978, 379(2): 103-112. DOI: 10.1007/BF00432479.
[12]	GORDON SC, WU KH, LINDOR K, et al. Ursodeoxycholic acid treatmentpreferentially improves overall survival among African Americans with primary biliary cholangitis[J]. Am J Gastroenterol, 2020, 115(2): 262-270. DOI: 10.14309/ajg.0000000000000512.
[13]	ANGULO P, DICKSON ER, THERNEAU TM, et al. Comparison of three doses of ursodeoxycholic acid in the treatment of primary biliary cirrhosis: A randomized trial[J]. J Hepatol, 1999, 30(5): 830-835. DOI: 10.1016/s0168-8278(99)80136-6.
[14]	ANGULO P, JORGENSEN RA, LINDOR KD. Incomplete response to ursodeoxycholic acid in primary biliary cirrhosis: Is a double dosage worthwhile?[J]. Am J Gastroenterol, 2001, 96(11): 3152-3157. DOI: 10.1111/j.1572-0241.2001.05270.x.
[15]	LINDOR KD, KOWDLEY KV, LUKETIC VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis[J]. Hepatology, 2009, 50(3): 808-814. DOI: 10.1002/hep.23082.
[16]	CHEN S, DUAN W, YOU H, et al. A brief review on prognostic models of primary biliary cholangitis[J]. Hepatol Int, 2017, 11(5): 412-418. DOI: 10.1007/s12072-017-9819-9.
[17]	CORPECHOT C, ABENAVOLI L, RABAHI N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis[J]. Hepatology, 2008, 48(3): 871-877. DOI: 10.1002/hep.22428.
[18]	CORPECHOT C, CHAZOUILLÈRES O, POUPON R. Early primary biliary cirrhosis: Biochemical response to treatment and prediction of long-term outcome[J]. J Hepatol, 2011, 55(6): 1361-1367. DOI: 10.1016/j.jhep.2011.02.031.
[19]	NEVENS F, ANDREONE P, MAZZELLA G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis[J]. N Engl J Med, 2016, 375(7): 631-643. DOI: 10.1056/NEJMoa1509840.
[20]	HIRSCHFIELD GM, BEUERS U, KUPCINSKAS L, et al. A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA[J]. J Hepatol, 2021, 74(2): 321-329. DOI: 10.1016/j.jhep.2020.09.011.
[21]	ZHANG LN, SHI TY, SHI XH, et al. Early biochemical response to ursodeoxycholic acid and long-term prognosis of primary biliary cirrhosis: Results of a 14-year cohort study[J]. Hepatology, 2013, 58(1): 264-272. DOI: 10.1002/hep.26322.
[22]	MANNE V, KOWDLEY KV. Obeticholic acid in primary biliary cholangitis: Where we stand[J]. Curr Opin Gastroenterol, 2019, 35(3): 191-196. DOI: 10.1097/MOG.0000000000000525.
[23]	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[J]. Lancet Gastroenterol Hepatol, 2019, 4(6): 445-453. DOI: 10.1016/S2468-1253(19)30094-9.
[24]	PARÉS A, SHIFFMAN M, VARGAS V, et al. Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis[J]. Liver Int, 2020, 40(5): 1121-1129. DOI: 10.1111/liv.14429.
[25]	BOWLUS CL, POCKROS PJ, KREMER AE, et al. Long-term obeticholic acid therapy improves histological endpoints in patients with primary biliary cholangitis[J]. Clin Gastroenterol Hepatol, 2020, 18(5): 1170-1178. e6. DOI: 10.1016/j.cgh.2019.09.050.
[26]	KOWDLEY KV, LUKETIC V, CHAPMAN R, et al. A randomized trial of obeticholic acid monotherapy in patients with primary biliary cholangitis[J]. Hepatology, 2018, 67(5): 1890-1902. DOI: 10.1002/hep.29569.
[27]	JOHN BV, SCHWARTZ K, LEVY C, et al. Impact of obeticholic acid exposure on decompensation and mortality in primary biliary cholangitis and cirrhosis[J]. Hepatol Commun, 2021, 5(8): 1426-1436. DOI: 10.1002/hep4.1720.
[28]	LINDOR KD, BOWLUS CL, BOYER J, et al. Primary biliary cholangitis: 2021 Practice Guidance Update from the American Association for the Study of Liver Diseases[J]. Hepatology, 2021. DOI: 10.1002/hep.32117.
[29]	ZHANG Y, LI S, HE L, et al. Combination therapy of fenofibrate and ursodeoxycholic acid in patients with primary biliary cirrhosis who respond incompletely to UDCA monotherapy: A meta-analysis[J]. Drug Des Devel Ther, 2015, 9: 2757-2766. DOI: 10.2147/DDDT.S79837.
[30]	CORPECHOT C, CHAZOUILLÈRES O, ROUSSEAU A, et al. A placebo-controlled trial of bezafibrate in primary biliary cholangitis[J]. N Engl J Med, 2018, 378(23): 2171-2181. DOI: 10.1056/NEJMoa1714519.
[31]	de VRIES E, BOLIER R, GOET J, et al. Fibrates for Itch (FITCH) in fibrosing cholangiopathies: A double-blind, randomized, placebo-controlled trial[J]. Gastroenterology, 2021, 160(3): 734-743. e6. DOI: 10.1053/j.gastro.2020.10.001.
[32]	TANAKA A, HIROHARA J, NAKANO T, et al. Association of bezafibrate with transplant-free survival in patients with primary biliary cholangitis[J]. J Hepatol, 2021, 75(3): 565-571. DOI: 10.1016/j.jhep.2021.04.010.
[33]	CARRION AF, LINDOR KD, LEVY C. Safety of fibrates in cholestatic liver diseases[J]. Liver Int, 2021, 41(6): 1335-1343. DOI: 10.1111/liv.14871.
[34]	DOHMEN K, TANAKA H, HARUNO M. Effectiveness of fenofibrate in comparison to bezafibrate for patients with asymptomatic primary biliary cirrhosis[J]. Fukuoka Igaku Zasshi, 2013, 104(10): 350-361. http://catalog.lib.kyushu-u.ac.jp/handle/2324/1398604/p350.pdf
[35]	WANG L, SUN K, TIAN A, et al. Fenofibrate improves GLOBE and UK-PBC scores and histological features in primary biliary cholangitis[J]. Minerva Med, 2021. DOI: 10.23736/S0026-4806.21.07316-X.[Onlineaheadofprint]
[36]	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[J]. Hepatology, 2005, 41(4): 747-752. DOI: 10.1002/hep.20646.
[37]	LEUSCHNER M, MAIER KP, SCHLICHTING J, et al. Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: Results of a prospective double-blind trial[J]. Gastroenterology, 1999, 117(4): 918-925. DOI: 10.1016/s0016-5085(99)70351-3.
[38]	HIRSCHFIELD GM, BEUERS U, KUPCINSKAS L, et al. A placebo-controlled randomised trial of budesonide for PBC following an insufficient response to UDCA[J]. J Hepatol, 2021, 74(2): 321-329. DOI: 10.1016/j.jhep.2020.09.011.
[39]	HEMPFLING W, GRUNHAGE F, DILGER K, et al. Pharmacokinetics and pharmacodynamic action of budesonide in early- and late-stage primary biliary cirrhosis[J]. Hepatology, 2003, 38(1): 196-202. DOI: 10.1053/jhep.2003.50266.
[40]	European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis[J]. J Hepatol, 2017, 67(1): 145-172. DOI: 10.1016/j.jhep.2017.03.022.
[41]	AGUILAR MT, CAREY EJ. Current status of liver transplantation for primary biliary cholangitis[J]. Clin Liver Dis, 2018, 22(3): 613-624. DOI: 10.1016/j.cld.2018.03.011.
[42]	MONTANO-LOZA AJ, HANSEN BE, CORPECHOT C, et al. Factors associated with recurrence of primary biliary cholangitis after liver transplantation and effects on graft and patient survival[J]. Gastroenterology, 2019, 156(1): 96-107. e1. DOI: 10.1053/j.gastro.2018.10.001.
[43]	SILVEIRA MG, TALWALKAR JA, LINDOR KD, et al. Recurrent primary biliary cirrhosis after liver transplantation[J]. Am J Transplant, 2010, 10(4): 720-726. DOI: 10.1111/j.1600-6143.2010.03038.x.
[44]	MONTANO-LOZA AJ, HANSEN BE, CORPECHOT C, et al. Factors associated with recurrence of primary biliary cholangitis after liver transplantation and effects on graft and patient survival[J]. Gastroenterology, 2019, 156(1): 96-107. e1. DOI: 10.1053/j.gastro.2018.10.001.
[45]	JACOB DA, NEUMANN UP, BAHRA M, et al. Liver transplantation for primary biliary cirrhosis: Influence of primary immunosuppression on survival[J]. Transplant Proc, 2005, 37(4): 1691-1692. DOI: 10.1016/j.transproceed.2005.03.130.
[46]	CORPECHOT C, CHAZOUILLÈRES O, BELNOU P, et al. Long-term impact of preventive UDCA therapy after transplantation for primary biliary cholangitis[J]. J Hepatol, 2020, 73(3): 559-565. DOI: 10.1016/j.jhep.2020.03.043.
[47]	CARBONE M, BUFTON S, MONACO A, et al. The effect of liver transplantation on fatigue in patients with primary biliary cirrhosis: A prospective study[J]. J Hepatol, 2013, 59(3): 490-494. DOI: 10.1016/j.jhep.2013.04.017.
[48]	SILVEIRA MG, GOSSARD AA, STAHLER AC, et al. A randomized, placebo-controlled clinical trial of efficacy and safety: Modafinil in the treatment of fatigue in patients with primary biliary cirrhosis[J]. Am J Ther, 2017, 24(2): e167-e176. DOI: 10.1097/MJT.0000000000000387.
[49]	SHAH RA, KOWDLEY KV. Mechanisms and treatments of pruritus in primary biliary cholangitis[J]. Semin Liver Dis, 2019, 39(2): 209-220. DOI: 10.1055/s-0039-1679918.
[50]	SCHAFFNER F, KLION FM, LATUFF AJ. The long term use of cholestyramine in the treatment of primary biliary cirrhosis[J]. Gastroenterology, 1965, 48: 293-298. http://europepmc.org/abstract/MED/14292140
[51]	TANDON P, ROWE BH, VANDERMEER B, et al. The efficacy and safety of bile acid binding agents, opioid antagonists, or rifampin in the treatment of cholestasis-associated pruritus[J]. Am J Gastroenterol, 2007, 102(7): 1528-1536. DOI: 10.1111/j.1572-0241.2007.01200.x.
[52]	ATAEI S, KORD L, LARKI A, et al. Comparison of sertraline with rifampin in the treatment of cholestatic pruritus: A randomized clinical trial[J]. Rev Recent Clin Trials, 2019, 14(3): 217-223. DOI: 10.2174/1574887114666190328130720.
[53]	JONES DE, NEWTON JL. An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis[J]. Aliment Pharmacol Ther, 2007, 25(4): 471-476. DOI: 10.1111/j.1365-2036.2006.03223.x.
[54]	HEGADE VS, PECHLIVANIS A, MCDONALD J, et al. Autotaxin, bile acid profile and effect of ileal bile acid transporter inhibition in primary biliary cholangitis patients with pruritus[J]. Liver Int, 2019, 39(5): 967-975. DOI: 10.1111/liv.14069.
[55]	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[J]. Sci Rep, 2018, 8(1): 6658. DOI: 10.1038/s41598-018-25214-0.
[56]	TATLIPINAR S, AKPEK EK. Topical ciclosporin in the treatment of ocular surface disorders[J]. Br J Ophthalmol, 2005, 89(10): 1363-1367. DOI: 10.1136/bjo.2005.070888.
[57]	VITALI C, MINNITI A, PIGNATARO F, et al. Management of Sjögren's Syndrome: Present issues and future perspectives[J]. Front Med (Lausanne), 2021, 8: 676885. DOI: 10.3389/fmed.2021.676885.
[58]	SEKI A, IKEDA F, MIYATAKE H, et al. Risk of secondary osteoporosis due to lobular cholestasis in non-cirrhotic primary biliary cholangitis[J]. J Gastroenterol Hepatol, 2017, 32(9): 1611-1616. DOI: 10.1111/jgh.13746.
[59]	TRIVEDI HD, DANFORD CJ, GOYES D, et al. Osteoporosis in primary biliary cholangitis: Prevalence, impact and management challenges[J]. Clin Exp Gastroenterol, 2020, 13: 17-24. DOI: 10.2147/CEG.S204638.
[60]	RUDIC JS, GILJACA V, KRSTIC MN, et al. Bisphosphonates for osteoporosis in primary biliary cirrhosis[J]. Cochrane Database Syst Rev, 2011, (12): CD009144. DOI: 10.1002/14651858.CD009144.pub2.
[61]	DANFORD CJ, EZAZ G, TRIVEDI HD, et al. The pharmacologic management of osteoporosis in primary biliary cholangitis: A systematic review and meta-analysis[J]. J Clin Densitom, 2020, 23(2): 223-236. DOI: 10.1016/j.jocd.2019.05.003.
[62]	GUAÑABENS N, MONEGAL A, CERDÁ D, et al. Randomized trial comparing monthly ibandronate and weekly alendronate for osteoporosis in patients with primary biliary cirrhosis[J]. Hepatology, 2013, 58(6): 2070-2078. DOI: 10.1002/hep.26466.
[63]	WANG Z, PENG C, WANG P, et al. Serum vitamin D level is related to disease progression in primary biliary cholangitis[J]. Scand J Gastroenterol, 2020, 55(11): 1333-1340. DOI: 10.1080/00365521.2020.1829030.
[64]	European Association for the Study of the Liver. EASL Clinical Practice Guidelines on nutrition in chronic liver disease[J]. J Hepatol, 2019, 70(1): 172-193. DOI: 10.1016/j.jhep.2018.06.024.
[65]	LINDOR KD, BOWLUS CL, BOYER J, et al. Primary biliary cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases[J]. Hepatology, 2019, 69(1): 394-419. DOI: 10.1002/hep.30145.
[66]	KANIS JA, COOPER C, RIZZOLI R, et al. European guidance for the diagnosis and management of osteoporosis in postmenopausal women[J]. Osteoporos Int, 2019, 30(1): 3-44. DOI: 10.1007/s00198-018-4704-5.
[67]	FLOREANI A, ZAPPALA F, FRIES W, et al. A 3-year pilot study with 1, 25-dihydroxyvitamin D, calcium, and calcitonin for severe osteodystrophy in primary biliary cirrhosis[J]. J Clin Gastroenterol, 1997, 24(4): 239-244. DOI: 10.1097/00004836-199706000-00012.
[68]	GATSELIS NK, DALEKOS GN. Molecular diagnostic testing for primary biliary cholangitis[J]. Expert Rev Mol Diagn, 2016, 16(9): 1001-1010. DOI: 10.1080/14737159.2016.1217159.
[69]	CHEN S, DUAN W, LI M, et al. Prognosis of 732 ursodeoxycholic acid-treated patients with primary biliary cholangitis: A single center follow-up study from China[J]. J Gastroenterol Hepatol, 2019, 34(7): 1236-1241. DOI: 10.1111/jgh.14521.
[70]	INVERNIZZI P, CROSIGNANI A, BATTEZZATI PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis[J]. Hepatology, 1997, 25(5): 1090-1095. DOI: 10.1002/hep.510250507.
[71]	JULIUSSON G, IMAM M, BJÖRNSSON ES, et al. Long-term outcomes in antimitochondrial antibody negative primary biliary cirrhosis[J]. Scand J Gastroenterol, 2016, 51(6): 745-752. DOI: 10.3109/00365521.2015.1132337.
[72]	SAKAUCHI F, MORI M, ZENIYA M, et al. Antimitochondrial antibody negative primary biliary cirrhosis in Japan: Utilization of clinical data when patients applied to receive public financial aid[J]. J Epidemiol, 2006, 16(1): 30-34. DOI: 10.2188/jea.16.30.
[73]	HIRSCHFIELD GM, HEATHCOTE EJ. Antimitochondrial antibody-negative primary biliary cirrhosis[J]. Clin Liver Dis, 2008, 12(2): 323-331; viii-ix. DOI: 10.1016/j.cld.2008.02.003.
[74]	JIN Q, MORITOKI Y, LLEO A, et al. Comparative analysis of portal cell infiltrates in antimitochondrial autoantibody-positive versus antimitochondrial autoantibody- negative primary biliary cirrhosis[J]. Hepatology, 2012, 55(5): 1495-1506. DOI: 10.1002/hep.25511.
[75]	DAHLQVIST G, GAOUAR F, CARRAT F, et al. Large-scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis[J]. Hepatology, 2017, 65(1): 152-163. DOI: 10.1002/hep.28859.
[76]	ZANDANELL S, STRASSER M, FELDMAN A, et al. Low rate of new-onset primary biliary cholangitis in a cohort of anti-mitochondrial antibody-positive subjects over six years of follow-up[J]. J Intern Med, 2020, 287(4): 395-404. DOI: 10.1111/joim.13005.
[77]	GULAMHUSEIN AF, JURAN BD, ATKINSON EJ, et al. Low incidence of primary biliary cirrhosis (PBC) in the first-degree relatives of PBC probands after 8 years of follow-up[J]. Liver Int, 2016, 36(9): 1378-1382. DOI: 10.1111/Liv.13143.
[78]	SUN C, XIAO X, YAN L, et al. Histologically proven AMA positive primary biliary cholangitis but normal serum alkaline phosphatase: Is alkaline phosphatase truly a surrogate marker?[J]. J Autoimmun, 2019, 99: 33-38. DOI: 10.1016/j.jaut.2019.01.005.
[79]	TERZIROLI BERETTA-PICCOLI B, STIRNIMANN G, MERTENS J, et al. Primary biliary cholangitis with normal alkaline phosphatase: A neglected clinical entity challenging current guidelines[J]. J Autoimmun, 2021, 116: 102578. DOI: 10.1016/j.jaut.2020.102578.
[80]	LOHSE AW, ZUM BVSCHENFELDE KH, FRANZ B, et al. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: Evidence for it being a hepatitic form of PBC in genetically susceptible individuals[J]. Hepatology, 1999, 29(4): 1078-1084. DOI: 10.1002/hep.510290409.
[81]	LEE BT, WANG Y, YANG A, et al. IgG: IgM ratios of liver plasma cells reveal similar phenotypes of primary biliary cholangitis with and without features of autoimmune hepatitis[J]. Clin Gastroenterol Hepatol, 2021, 19(2): 397-399. DOI: 10.1016/j.cgh.2019.11.024.
[82]	CHAZOUILLÈRES O, WENDUM D, SERFATY L, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: Clinical features and response to therapy[J]. Hepatology, 1998, 28(2): 296-301. DOI: 10.1002/hep.510280203.
[83]	YANG F, WANG Q, BIAN Z, et al. Autoimmune hepatitis: East meets west[J]. J Gastroenterol Hepatol, 2015, 30(8): 1230-1236. DOI: 10.1111/jgh.12952.
[84]	WANG Q, SELMI C, ZHOU X, et al. Epigenetic considerations and the clinical reevaluation of the overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis[J]. J Autoimmun, 2013, 41: 140-145. DOI: 10.1016/j.jaut.2012.10.004.
[85]	MURATORI P, GRANITO A, PAPPAS G, et al. The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome[J]. Am J Gastroenterol, 2009, 104(6): 1420-1425. DOI: 10.1038/ajg.2009.126.
[86]	EFE C, PURNAK T, OZASLAN E, et al. The serological profile of the autoimmune hepatitis/primary biliary cirrhosis overlap syndrome[J]. Am J Gastroenterol, 2010, 105(1): 226; author reply 226-227. DOI: 10.1038/ajg.2009.602.
[87]	BOBERG KM, CHAPMAN RW, HIRSCHFIELD GM, et al. Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue[J]. J Hepatol, 2011, 54(2): 374-385. DOI: 10.1016/j.jhep.2010.09.002.
[88]	KUIPER EM, ZONDERVAN PE, VAN BUUREN HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome[J]. Clin Gastroenterol Hepatol, 2010, 8(6): 530-534. DOI: 10.1016/j.cgh.2010.03.004.
[89]	YANG F, WANG Q, WANG Z, et al. The natural history and prognosis of primary biliary cirrhosis with clinical features of autoimmune hepatitis[J]. Clin Rev Allergy Immunol, 2016, 50(1): 114-123. DOI: 10.1007/s12016-015-8516-5.
[90]	FREEDMAN BL, DANFORD CJ, PATWARDHAN V, et al. Treatment of overlap syndromes in autoimmune liver disease: A systematic review and meta-analysis[J]. J Clin Med, 2020, 9(5). DOI: 10.3390/jcm9051449.
[91]	ZHANG H, LI S, YANG J, et al. A meta-analysis of ursodeoxycholic acid therapy versus combination therapy with corticosteroids for PBC-AIH-overlap syndrome: Evidence from 97 monotherapy and 117 combinations[J]. Prz Gastroenterol, 2015, 10(3): 148-155. DOI: 10.5114/pg.2015.51187.
[92]	OZASLAN E, EFE C, HEURGUÉ-BERLOT A, et al. Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis[J]. Clin Gastroenterol Hepatol, 2014, 12(5): 863-869. DOI: 10.1016/j.cgh.2013.09.021.
[93]	MAGO S, WU GY. Primary sclerosing cholangitis and primary biliary cirrhosis overlap syndrome: A review[J]. J Clin Transl Hepatol, 2020, 8(3): 336-346. DOI: 10.14218/JCTH.2020.00036.
[94]	FLOREANI A, INFANTOLINO C, FRANCESCHET I, et al. Pregnancy and primary biliary cirrhosis: A case-control study[J]. Clin Rev Allergy Immunol, 2015, 48(2-3): 236-242. DOI: 10.1007/s12016-014-8433-z.
[95]	TRIVEDI PJ, KUMAGI T, AL-HARTHY N, et al. Good maternal and fetal outcomes for pregnant women with primary biliary cirrhosis[J]. Clin Gastroenterol Hepatol, 2014, 12(7): 1179-1185. e1. DOI: 10.1016/j.cgh.2013.11.030.
[96]	CAULDWELL M, MACKIE FL, STEER PJ, et al. Pregnancy outcomes in women with primary biliary cholangitis and primary sclerosing cholangitis: A retrospective cohort study[J]. BJOG, 2020, 127(7): 876-884. DOI: 10.1111/1471-0528.16119.
[97]	EFE C, KAHRAMANO G Ǧ LU-AKSOY E, YILMAZ B, et al. Pregnancy in women with primary biliary cirrhosis[J]. Autoimmun Rev, 2014, 13(9): 931-935. DOI: 10.1016/j.autrev.2014.05.008.
[98]	HEMPFLING W, DILGER K, BEUERS U. Systematic review: Ursodeoxycholic acid-adverse effects and drug interactions[J]. Aliment Pharmacol Ther, 2003, 18(10): 963-972. DOI: 10.1046/j.1365-2036.2003.01792.x.
[99]	OVADIA C, SAJOUS J, SEED PT, et al. Ursodeoxycholic acid in intrahepatic cholestasis of pregnancy: A systematic review and individual participant data meta-analysis[J]. Lancet Gastroenterol Hepatol, 2021, 6(7): 547-558. DOI: 10.1016/S2468-1253(21)00074-1.
[100]	RUDI J, SCHÖNIG T, STREMMEL W. Therapy with ursodeoxycholic acid in primary biliary cirrhosis in pregnancy[J]. Z Gastroenterol, 1996, 34(3): 188-191.
[101]	VÍTEK L, ZELENKOVÁ M, BRǓHA R. Safe use of ursodeoxycholic acid in a breast-feeding patient with primary biliary cirrhosis[J]. Dig Liver Dis, 2010, 42(12): 911-912. DOI: 10.1016/j.dld.2010.06.002.
[102]	BRITES D, RODRIGUES CM. Elevated levels of bile acids in colostrum of patients with cholestasis of pregnancy are decreased following ursodeoxycholic acid therapy[J]. J Hepatol, 1998, 29(5): 743-751. DOI: 10.1016/s0168-8278(98)80255-9.
[103]	FAN X, WANG T, SHEN Y, et al. Underestimated male prevalence of primary biliary cholangitis in China: Results of a 16-yr cohort study involving 769 patients[J]. Sci Rep, 2017, 7(1): 6560. DOI: 10.1038/s41598-017-06807-7.
[104]	NATARAJAN Y, TANSEL A, PATEL P, et al. Incidence of hepatocellular carcinoma in primary biliary cholangitis: A systematic review and meta-analysis[J]. Dig Dis Sci, 2021, 66(7): 2439-2451. DOI: 10.1007/s10620-020-06498-7.
[105]	NAKAMURA M, KONDO H, TANAKA A, et al. Autoantibody status and histological variables influence biochemical response to treatment and long-term outcomes in Japanese patients with primary biliary cirrhosis[J]. Hepatol Res, 2015, 45(8): 846-855. DOI: 10.1111/hepr.12423.
[106]	POUPON R, CHAZOUILLÉRES O, BALKAU B, et al. Clinical and biochemical expression of the histopathological lesions of primary biliary cirrhosis. UDCA-PBC Group[J]. J Hepatol, 1999, 30(3): 408-412. DOI: 10.1016/s0168-8278(99)80098-1.
[107]	NAKANUMA Y, HOSO M, MIZUNO Y, et al. Pathologic study of primary biliary cirrhosis of early histologic stages presenting cholestatic jaundice[J]. Liver, 1988, 8(6): 319-324. DOI: 10.1111/j.1600-0676.1988.tb01010.x.
[108]	HARADA K, HSU M, IKEDA H, et al. Application and validation of a new histologic staging and grading system for primary biliary cirrhosis[J]. J Clin Gastroenterol, 2013, 47(2): 174-181. DOI: 10.1097/MCG.0b013e31827234e4.
[109]	LAMMERS WJ, HIRSCHFIELD GM, CORPECHOT C, et al. Development and validation of a scoring system to predict outcomes of patients with primary biliary cirrhosis receiving ursodeoxycholic acid therapy[J]. Gastroenterology, 2015, 149(7): 1804-1812. e4. DOI: 10.1053/j.gastro.2015.07.061.
[110]	CARBONE M, SHARP SJ, FLACK S, et al. The UK-PBC risk scores: Derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis[J]. Hepatology, 2016, 63(3): 930-950. DOI: 10.1002/hep.28017.
[111]	YANG F, YANG Y, WANG Q, et al. The risk predictive values of UK-PBC and GLOBE scoring system in Chinese patients with primary biliary cholangitis: The additional effect of anti-gp210[J]. Aliment Pharmacol Ther, 2017, 45(5): 733-743. DOI: 10.1111/apt.13927.
[112]	EFE C, TAȘÇILAR K, HENRIKSSON I, et al. Validation of risk scoring systems in ursodeoxycholic acid-treated patients with primary biliary cholangitis[J]. Am J Gastroenterol, 2019, 114(7): 1101-1108. DOI: 10.14309/ajg.0000000000000290.
[113]	LAZARIDIS KN, JURAN BD, BOE GM, et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis[J]. Hepatology, 2007, 46(3): 785-792. DOI: 10.1002/hep.21749.

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Guidelines on the diagnosis and management of primary biliary cholangitis (2021)

DOI: 10.3760/cma.j.cn112138-20211112-00794

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Guidelines on the diagnosis and management of primary biliary cholangitis (2021)

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