原发性胆汁性肝硬化预后判断及其治疗方案

黄春洋; 刘燕敏

doi:10.3969/j.issn.1001-5256.2016.07.009

原发性胆汁性肝硬化预后判断及其治疗方案

DOI: 10.3969/j.issn.1001-5256.2016.07.009

首都医科大学附属北京佑安医院

基金项目:

北京市丰台区卫生系统科学研究项目(2014-19)；首都卫生发展科研专项青年项目(首发2014-4-2183)；

详细信息

中图分类号: R575.22
计量
- 文章访问数: 2142
- HTML全文浏览量: 14
- PDF下载量: 505
- 被引次数: 0
出版历程
- 收稿日期: 2016-05-03
- 出版日期: 2016-07-20

Prognostic evaluation of primary biliary cirrhosis and its value in guiding therapeutic regimens

Beijing You An Hospital,Capital Medical University

Research funding:

摘要

摘要: 原发性胆汁性肝硬化(PBC)患者的预后评判以及如何改善预后一直备受关注,预后不佳PBC患者的进一步治疗方案成为临床及科研的研究方向。归纳了PBC患者基线指标与预后的关系及熊去氧胆酸(UDCA)治疗中患者预后的评价,介绍了对UDCA应答不佳患者联合布地耐德、贝特类药物及奥贝胆酸的治疗现状,以及正在研究开发的药物,分析了可能影响预后和UDCA疗效的其他因素。认为PBC患者UDCA治疗前及治疗中需对预后及疗效进行评价,及时调整治疗方案,改善预后。
- 肝硬化,胆汁性 /
- 治疗 /
- 预后
Abstract: Prognostic evaluation of patients with primary biliary cirrhosis( PBC) and how to improve the prognosis have attracted much attention. Further therapeutic regimens for PBC patients with poor prognosis has become the direction of clinical and scientific studies. This article summarizes the association between baseline indices and prognosis and prognostic evaluation of patients undergoing ursodeoxycholic acid( UDCA) treatment,introduces the current status of UDCA combined with budesonide,fibrates,and obeticholic acid for patients with poor response to UDCA and the drugs being developed,and analyzes the influencing factors for prognosis and efficacy of UDCA. It is pointed out that prognosis and efficacy should be evaluated before and during UDCA treatment,and that therapeutic regimens should be adjusted in time to improve prognosis.
- liver cirrhosis /
- biliary /
- therapy

HTML全文

参考文献(41)

[1]BEUERS U,GERSHWIN ME,GISH RG,et al.Changing nomenclature for PBC:from‘cirrhosis’to‘cholangitis’[J].Gut,2015,64(11):1671-1672.

[2]SHAPIRO JM,SMITH H,SCHAFFNER F.Serum bilirubin:a prognostic factor in primary biliary cirrhosis[J].Gut,1979,20(2):137-140.

[3]ROLL J,BOYER JL,BARRY D,et al.The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis[J].N Engl J Med,1983,308(1):1-7.

[4]CHRISTENSEN E,NEUBERGER J,CROWE J,et al.Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis.Final results of an international trial[J].Gastroenterology,1985,89(5):1084-1091.

[5]DICKSON ER,GRAMBSCH PM,FLEMING TR,et al.Prognosis in primary biliary cirrhosis:model for decision making[J].Hepatology 1989,10(1):1-7.

[6]PRINCE M,CHETWYND A,NEWMAN W,et al.Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis:follow-up for up to 28 years[J].Gastroenterology,2002,123(4):1044-1051.

[7]HEATHCOTE EJ.Management of primary biliary cirrhosis.The American Association for the Study of Liver Diseases practice guidelines[J].Hepatology,2000,31(4):1005-1013.

[8]ANGULO P,LINDOR KD,THERNEAU TM,et al.Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid[J].Liver,1999,19(2):115-121.

[9]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.

[10]CHAN AW,CHAN RC,WONG GL,et al.New simple prognostic score for primary biliary cirrhosis:albumin-bilirubin score[J].JGastroenterol Hepatol,2015,30(9):1391-1396.

[11]JOHNSON PJ,BERHANE S,KAGEBAYASHI C,et al.Assessment of liver function in patients with hepatocellular carcinoma:a new evidence-based approach-the ALBI grade[J].J Clin Oncol,2015,33(6):550-558.

[12]KUMAGI T,GUINDI M,FISCHER SE,et al.Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis[J].Am J Gastroenterol,2010,105(10):2186-2194.

[13]CORPECHOT C,CHAZOUILLRES 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.

[14]AZEMOTO N,ABE M,MURATA Y,et al.Early biochemical response to ursodeoxycholic acid predicts symptom development in patients with asymptomatic primary biliary cirrhosis[J].J Gastroenterol,2009,44(6):630-634.

[15]LAMMERS WJ,van BUUREN HR,HIRSCHFIELD GM,et al.Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis:an international follow-up study[J].Gastroenterology,2014,147(6):1338-1349.

[16]PARS A,CABALLERA L,RODS J.Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid[J].Gastroenterology,2006,130(3):715-720.

[17]KUIPER EM,HANSEN BE,de VRIES RA,et al.Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid[J].Gastroenterology,2009,136(4):1281-1287.

[18]FLOREANI A,CAZZAGON N,MARTINES D,et al.Performance and utility of transient elastography and noninvasive markers of liver fibrosis in primary biliary cirrhosis[J].Dig Liver Dis,2011,43(11):887-892.

[19]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.

[20]DUAN WJ,ZHANG FK,OU XJ,et al.The clinical profiles of primary biliary cirrhosis with a suboptimal biochemical response to ursodeoxycholic acid[J].Chin J Hepatol,2011,19(2):118-120.(in Chinese)段维佳,张福奎,欧晓娟,等.原发性胆汁性肝硬化患者对熊去氧胆酸应答欠佳的影响因素分析[J].中华肝脏病杂志,2011,19(2):118-120.

[21]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.

[22]LAMMERSWJ,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.

[23]Chinese Society of Hepatology,Chinese Medical Association;Chinese Society of Gastroenterology,Chinese Medical Association;Chinese Society of Infectious Diseases,Chinese Medical Association.Consensus 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.

[24]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.

[25]ANGULO P,JORGENSEN RA,KEACH JC,et al.Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid[J].Hepatology,2000,31(2):318-323.

[26]RAUTIAINEN H,KRKKINEN 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.

[27]RABAHI N,CHRTIEN Y,GAOUAR F,et al.Triple therapy with ursodeoxycholic acid,budesonide and mycophenolate mofetil in patients with features of severe primary biliary cirrhosis not responding to ursodeoxycholic acid alone[J].Gastroenterol Clin Biol,2010,34(4-5):283-287.

[28]RAUTIAINEN H,KARVONEN AL,KARKKAINEN P,et al.6year follow up of liver histology after budesonide to UDCA combination therapy in PBC compared with UDCA monoterapy[J].J Hepatol,2011,54(Suppl):s517.

[29]RAUTIAINEN H,FRKKILM,NEUVONEN M,et al.Pharmacokinetics and bone effects of budesonide in primary biliary cirrhosis[J].Aliment Pharmacol Ther,2006,24(11-12):1545-1552.

[30]NEVENS F,ANDREONE P,MAZZELLA G,et al.The first primary biliary cirrhosis(PBC)phase 3 trial in two decades---an international study of the FXR agonist obeticholic acid[J].J Hepatol,2014,60:s525.

[31]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.

[32]IWASAKI S,OHIRA H,NISHIGUCHI S,et al.The efficacy of ursodeoxycholic acid and bezafibrate combination therapy for primary biliary cirrhosis:a prospective,multicenter study[J].Hepatol Res,2008,38(6):557-564.

[33]KANDA T,YOKOSUKA O,IMAZEKI F,et al.Bezafibrate treatment:a new medical approach for PBC patients?[J].J Gastroenterol,2003,38(6):573-578.

[34]ITAKURA J,IZUMI N,NISHIMURA Y,et al.Prospective randomized crossover trial of combination therapy with bezafibrate and UDCA for primary biliary cirrhosis[J].Hepatol Res,2004,29(4):216-222.

[35]LEVY C,PETER JA,NELSON DR,et al.Pilot study:fenofibrate for patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid[J].Aliment Pharmacol Ther,2011,33(2):235-242.

[36]LENS S,LEOZ M,NAZAL L,et al.Bezafibrate normalizes alkaline phosphatase in primary biliary cirrhosis patients with incomplete response to ursodeoxycholic acid[J].Liver Int,2014,34(2):197-203.

[37]CORPECHOT C.Primary biliary cirrhosis beyond ursodeoxycholic acid[J].Semin Liver Dis,2016,36(1):15-26.

[38]SUN LM,WANG YP,LIU YM,et al.Differential characteristics ofA-MA-M2 autoantibody in primary biliary cirrhosis and non-PBC patients[J].Chin J Hepatol,2015,23(5):343-349.(in Chinese)孙丽梅,王一鹏,刘燕敏,等.抗线粒体抗体M2亚型在非原发性胆汁性肝硬化患者中的特点[J].中华肝脏病杂志,2015,23(5):343-349.

[39]de LEMOS AS,FOUREAU DM,JACOBS C,et al.Drug-induced liver injury with autoimmune features[J].Semin Liver Dis,2014,34(2):194-204.

[40]LIU YM,LIAO HY,YAN HP,et al.A clinical study of bilirubin rise in short-term in patients with primary biliary cirrhosis[J].Chin J Hepatol,2012,20(8):632-633.(in Chinese)刘燕敏,廖慧钰,闫惠平,等.原发性胆汁性肝硬化患者胆红素短期内上升临床分析[J].中华肝脏病杂志,2012,20(8):632-633.

[41]European Association for the Study of the Liver.EASL clinical practice guidelines:management of cholestatic liver diseases[J].JHepatol,2009,51(2):237-267.

施引文献

资源附件(0)

访问统计