中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Issue 10
Oct.  2016
Turn off MathJax
Article Contents

Advances in diagnosis and treatment of primary biliary cholangitis

DOI: 10.3969/j.issn.1001-5256.2016.10.046
Research funding:

 

  • Published Date: 2016-10-20
  • Primary biliary cholangitis is an organ-specific chronic cholestatic autoimmune liver disease characterized by intrahepatic cholestasis,presence of anti-mitochondrial antibody in circulating blood,and progressive and non-suppurative damage of small intrahepatic bile ducts,which finally lead to extensive hepatic duct damage,biliary cirrhosis,and even liver failure. This article overviews the advances in the epidemiology,pathogenesis,diagnosis,and treatment of primary biliary cholangitis and points out that the safety and efficacy of the new drugs such as fibrates in the treatment of primary biliary cholangitis need to be confirmed by further studies.

     

  • loading
  • [1]ADDISON T,GULL W.On a certain affection of the skin:vitiligoidea-alpha,plana,beta tuberosa[J].Guy's Hosp Rep,1851,7:265-276.
    [2]Mac MAHON HE,THANNHAUSER SJ.Xanthomatous biliary cirrhosis:a clinical syndrome[J].Ann Intern Med,1949,30(1):121-179.
    [3]SHERLOCK S.Primary billiary cirrhosis(chronic intrahepatic obstructive jaundice)[J].Gastroenterology,1959,37:574-586.
    [4]BEUERS U,GERSHWIN ME,GISH RG,et al.Changing nomenclature for PBC:from‘cirrhosis’to‘cholangitis’[J].Hepatology,2015,62(5):1620-1622.
    [5]LIU C,XU CY,LI GD,et al.Analysis of clinical features of patients with autoimmune hepatitis/primary biliary cirrhosis overlap syndrome[J].J Jilin Univ:Med Edit,2014,40(3):646-649.(in Chinese)刘晨,徐长妍,李国东,等.自身免疫性肝炎/源发性胆汁性肝硬化重叠综合征患者临床特征分析[J].吉林大学学报:医学版,2014,40(3):646-649.
    [6]BOONSTRA K,BEUERS U,PONSIOEN CY.Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis:a systematic review[J].J Hepatol,2012,56(5):1181-1188.
    [7]GRIFFITHS L,DYSON JK,JONES DE.The new epidemiology of primary biliary cirrhosis[J].Semin Liver Dis,2014,34(3):318-328.
    [8]WATSON RG,ANGUS PW,DEWAR M,et al.Low prevalence of primary biliary cirrhosis in Victoria,Australia.Melbourne Liver Group[J].Gut,1995,36(6):927-930.
    [9]SMYK DS,RIGOPOULOU EI,BOGDANOS DP.Potential roles for infectious agents in the pathophysiology of primary biliary cirrhosis:what's new?[J].Curr Infect Dis Rep,2013,15(1):14-24.
    [10]MANTAKA A,KOULENTAKI M,CHLOUVERAKIS G,et al.Primary biliary cirrhosis in a genetically homogeneous population:disease associations and familial occurrence rates[J].BMC Gastroenterol,2012,12:110.
    [11]CORPECHOT C,CHRETIEN Y,CHAZOUILLERES O,et al.Demographic,lifestyle,medical and familial factors associated with primary biliary cirrhosis[J].J Hepatol,2010,53(1):162-169.
    [12]BEUERS U,TRAUNER M,JANSEN P,et al.New paradigms in the treatment of hepatic cholestasis:from UDCA to FXR,PXR and beyond[J].J Hepatol,2015,62(1 Suppl):s25-s37.
    [13]Chinese Society of Hepatology,Chinese Medical Association,Chinese Society of Gastroenterology,Chinese Medical Association,Chinese Socitety of Infctious,Chinese Medical Associety.Consensous on the diagnosis and management of primary biliary cirrhosis(cholangitis)(2015)[J].J Clin Hepatol,2015,31(12):1980-1988.(in Chinese)中华医学会肝病学分会,中华医学会消化病学分会,中华医学会感染病学分会.原发性胆汁性肝硬化(又名原发性胆汁性胆管炎)诊断和治疗共识(2015)[J].临床肝胆病杂志,2015,31(12):1980-1988.
    [14]YAMAGIWA S,KAMIMURA H,TAKAMURA M,et al.Autoantibodies in primary biliary cirrhosis:recent progress in research on the pathogenetic and clinical significance[J].World J Gastroentero,2014,20(10):2606-2612.
    [15]NAKAMURA M.Clinical significance of autoantibodies in primary biliary cirrhosis[J].Semin Liver Dis,2014,34(3):334-340.
    [16]INVERNIZZI P,SELMI C,RANFTLER C.et al.Antinuclear antibodies in primary biliary cirrhosis[J].Semin Liver Dis,2005,25(3):298-310.
    [17]IMAM MH,LINDOR KD.The natural history of primary biliary cirrhosis[J].Semin Liver Dis,2014,34(3):329-333.
    [18]DENG HM,HU SL,CHEN WX,et al.Meta-analysis of antiGP210 antibody and anti-SP100 antibody detection for diagnosis of primary biliary cirrhosis[J].Chin J Hepatol,2016,24(1):62-68.(in Chinese)邓红梅,胡世玲,陈维贤,等.抗核孔膜蛋白和抗核体蛋白抗体对原发性胆汁性肝硬化诊断价值的meta分析[J].中华肝脏病杂志,2016,24(1):62-68.
    [19]MITCHISON HC,BASSENDINE MF,HENDRICK A,et al.Positive antimitochondrial antibody but normal alkaline phosphatase——is this primary biliary cirrhosis?[J].Hepatology,1986,6:1279-1284.
    [20]SILVEIRA MG,TALWALKAR JA,ANGULO P,et al.Overlap of autoimmune hepatitis and primary biliary cirrhosis:long-term outcomes[J].Am J Gastroenterol,2007,102(6):1244-1250.
    [21]TAN S,MOVAHEDI Z,BEISEL J,et al.Correlation of anti-mitochondrial antibodies with liver histology and outcomes[J].Dig Dis Sci,2016,61(6):1770-1771.
    [22]LINDGREN S,GLAUMANN H,ALMER S,et al.Transitions between variant forms of primary biliary cirrhosis during long-term follow-up[J].Eur J Intern Med,2009,20(4):398-402.
    [23]JULIUSSON G,IMAM M,BJORNSSON ES,et al.Long-term outcomes in antimitochondrial antibody negative primary biliary cirrhosis[J].Scand J Gastroenterol,2016,51(6):745-752.
    [24]HIRSCHFIELD GM,HEATHCOTE EJ.Antimitochondrial antibody-negative primary biliary cirrhosis[J].Clin Liver Dis,2008,12(2):323-331;viii-ix.
    [25]PARES A,CABALLERIA L,RODES J.Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid[J].Gastroenterology,2006,130(3):715-720.
    [26]ter BORG PC,SCHALM SW,HANSEN BE,et al.Prognosis of ursodeoxycholic Acid-treated patients with primary biliary cirrhosis.Results of a 10-yr cohort study involving 297 patients[J].Am J Gastroenterol,2006,101(9):2044-2050.
    [27]POUPON RE,LINDOR KD,CAUCH-DUDEK K,et al.Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis[J].Gastroenterology,1997,113(3):884-890.
    [28]ABBAS G,LINDOR KD.Pharmacological treatment of biliary cirrhosis with ursodeoxycholic acid[J].Expert Opin Pharmacother,2010,11(3):387-392.
    [29]KUIPER EM,HANSEN BE,ADANG RP,et al.Relatively high risk for hepatocellular carcinoma in patients with primary biliary cirrhosis not responding to ursodeoxycholic acid[J].Eur J Gastroenterol Hepatol,2010,22:1495-1502.
    [30]CUPERUS FJ,HALILBASIC E,TRAUNER M.Fibrate treatment for primary biliary cirrhosis[J].Curr Opin Gastroenterol,2014,30(3):279-286.
    [31]HONDA A,IKEGAMI T,NAKAMUTA M,et al.Anticholestatic effects of bezafibrate in patients with primary biliary cirrhosis treated with ursodeoxycholic acid[J].Hepatology,2013,57(5):1931-1941.
    [32]HAN XF,WANG QX,LIU Y,et al.Efficacy of fenofibrate in Chinese patients with primary biliary cirrhosis partially responding to ursodeoxycholic acid therapy[J].J Dig Dis,2012,13(4):219-224.
    [33]GRIGORIAN AY,MARDINI HE,CORPECHOT C,et al.Fenofibrate is effective adjunctive therapy in the treatment of primary biliary cirrhosis:a meta-analysis[J].Clin Res Hepatol Gastroenterol,2015,39(3):296-306.
    [34]YIN Q,LI J,XIA Y,et al.Systematic review and meta-analysis:bezafibrate in patients with primary biliary cirrhosis[J].Drug Des Devel Ther,2015,9:5407-5419.
    [35]TSUDA M,MORITOKI Y,LIAN ZX,et al.Biochemical and immunologic effects of rituximab in patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid[J].Hepatology,2012,55(2):512-521.
    [36]MYERS RP,SWAIN MG,LEE SS,et al.B-cell depletion with rituximab in patients with primary biliary cirrhosis refractory to ursodeoxycholic acid[J].Am J Gastroenterol,2013,108(6):933-941.
    [37]YIN YF,ZHANG X.B cell depletion in treating primary biliary cirrhosis:pros and cons[J].World J Gastroenterol,2012,18(30):3938-3940.
    [38]HIRSCHFIELD GM,LIU X,XU C,et al.Primary biliary cirrhosis associated with HLA,IL12A,and IL12RB2 variants[J].N Engl J Med,2009,360(24):2544-2555.
    [39]HIRSCHFIELD GM,GERSHWIN ME,STRAUSS R,et al.Ustekinumab for patients with primary biliary cholangitis who have an inadequate response to ursodeoxycholic acid:a proof-of-concept study[J].Hepatology,2016,64(1):189-199.
    [40]HIRSCHFIELD GM,MASON A,LUKETIC V,et al.Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid[J].Gastroenterology,2015,148(4):751-761,e758.
    [41]TRIVEDI PJ,HIRSCHFIELD GM,GERSHWIN ME.Obeticholic acid for the treatment of primary biliary cirrhosis[J].Expert Rev Clin Pharmacol,2016,9(1):13-26.
    [42]ALI AH,TABIBIAN JH,CAREY EJ,et al.Emerging drugs for the treatment of primary biliary cholangitis[J].Expert Opin Emerg Drugs,2016,21(1):39-56.
    [43]RAUTIAINEN H,KARKKAINEN 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.
    [44]WANG L,HAN Q,CHEN H,et al.Allogeneic bone marrow mesenchymal stem cell transplantation in patients with UDCA-resistant primary biliary cirrhosis[J].Stem Cells Dev,2014,23(20):2482-2489.
    [45]WANG L,LI J,LIU H,et al.Pilot study of umbilical cord-derived mesenchymal stem cell transfusion in patients with primary biliary cirrhosis[J].J Gastroenterol Hepatol,2013,28(Suppl 1):85-92.
    [46]DU Y,FANG L.Clinical efficacy of traditional Chinese medicine/herbal decotion combined with ursodeoxycholic acid for primary biliary cirrhosis:a meta-analysis[J].J Clin Hepatol,2015,31(1):68-73.(in Chinese)杜莹,方蕾.中药汤剂/中成药联合熊去氧胆酸治疗原发性胆汁性肝硬化疗效的meta分析[J].临床肝胆病杂志,2015,31(1):68-73.
    [47]REN M,ZHENG YJ,ZHU JF.TCM syndrome differentiation type of primary biliary cirrhosis[J].Jilin J Tradit Chin Med,2016,36(7):678-681.(in Chinese)任朦,郑亚江,祝峻峰.原发性胆汁性胆汁性肝硬化中医辨证分型[J].吉林中医药,2016,36(7):678-681.
    [48]POUPON R.Evidence-based treatment of primary biliary cirrhosis[J].Dig Dis,2014,32(5):626-630.
    [49]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[J].Clin Gastroenterol Hepatol,2011,9(9):776-780.
    [50]RACZYNSKA J,HABIOR A,PACZEK L,et al.Primary biliary cirrhosis in the era of liver transplantation[J].Ann Transplant,2014,19:488-493.
    [51]European Association for the Study of the Liver.EASL clinical practice guidelines:management of cholestatic liver diseases[J].J Hepatol,2009,51(2):237-267.
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Article Metrics

    Article views (1404) PDF downloads(416) Cited by()
    Proportional views
    Related

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return