中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R

留言板

尊敬的读者、作者、审稿人, 关于本刊的投稿、审稿、编辑和出版的任何问题, 您可以本页添加留言。我们将尽快给您答复。谢谢您的支持!

姓名
邮箱
手机号码
标题
留言内容
验证码

原发性胆汁性胆管炎预后模型的演进与应用

万玲玲 梁丽娜 卢书明

引用本文:
Citation:

原发性胆汁性胆管炎预后模型的演进与应用

DOI: 10.12449/JCH250730
利益冲突声明:本文不存在任何利益冲突。
作者贡献声明:万玲玲负责收集、查阅、整理文献及论文书写;梁丽娜负责文献查阅指导及修改论文;卢书明负责设计、指导及论文审阅并最后定稿。
详细信息
    通信作者:

    卢书明, lsm2003@126.com (ORCID: 0000-0002-4048-0841)

Research advances in prognostic models for primary biliary cholangitis

More Information
    Corresponding author: LU Shuming, lsm2003@126.com (ORCID: 0000-0002-4048-0841)
  • 摘要: 原发性胆汁性胆管炎(PBC)是一种自身免疫性疾病,好发于中年女性。PBC早期临床症状不明显,最终可发展至肝硬化并出现各种并发症。熊去氧胆酸是治疗PBC最有效的药物,但仍有40%的PBC患者对熊去氧胆酸应答不敏感,导致病情缓慢进展。准确评估PBC患者病情及预后有助于治疗方案的优化。本文就PBC预后模型的研究进展进行综述,以期为临床治疗提供参考。

     

  • 表  1  PBC预后模型/评分系统

    Table  1.   Primary biliary cholangitis prognostic models and scoring systems

    预后模型/评分系统 模型组成/评分定义
    Yale模型 0.037×1.037×年龄+0.74×2.1×肝肿大(否=0,是=1)-1.34×0.26×门静脉纤维化(否=0,是=1)+0.82×2.26×[胆
    红素≥5 mg/dL(否=0,是=1)]-0.73×0.48×[胆红素<1.5 mg/dL(否=0,是=1)]
    简化版Mayo模型 0.871×lnTBil(mg/dL)-2.53×lnAlb(mg/dL)+0.039×年龄+2.38×ln凝血酶原时间(s)+0.859×水肿积分1)
    European模型 0.04×(年龄-55)+1.39×腹水(否=0,是=1)+0.65×静脉曲张破裂出血(否=0,是=1)-0.085×[Alb(g/L)-34.3]+
    2.53×[log胆红素(µmol/L)-1.53]
    Rochester标准 ALP<2×ULN和Mayo模型<4.5分
    巴塞罗那标准 ALP水平下降>40%或降至正常(UDCA治疗1年)
    ParisⅠ标准 ALP≤3×ULN,AST≤2×ULN,胆红素正常(UDCA治疗1年)
    鹿特丹标准 胆红素和/或Alb降至正常(UDCA治疗1年)
    爱媛标准 GGT水平下降≥70%或降至正常(UDCA治疗6个月)
    多伦多标准 ALP<1.67×ULN(UDCA治疗2年)
    Paris Ⅱ标准 ALP≤1.5×ULN,AST≤1.5×ULN,胆红素正常(UDCA治疗1年)
    APRI指数 APRI≤0.54(UDCA治疗前或治疗1年)
    ALBI评分 -0.085×Alb(g/L)+0.66×log胆红素(µmol/L)
    GLOBE评分 0.044 378×年龄+0.939 82×ln(胆红素×ULN)+0.335 648×ln(ALP×ULN)-2.266 708×(Alb× LLN)-0.002 581×
    血小板计数(109/L)+1.216 8652)
    UK-PBC评分 1-基线幸存者功能ˆexp{0.028 785 43×(治疗1年时ALP×ULN-1.722 136 304)-0.042 287 3×[(治疗1年时ALT
    或AST×ULN/10)-1-8.675 729 006]+1.419 9×[ln(治疗1年时胆红素×ULN/10)+2.709 607 778]-1.960 303×(基
    线Alb×LLN-1.176 730 01)-0.416 195 4×(基线血小板×LLN-1.873 564 875)}3)

    注:1)水肿积分(0,无水肿;0.5,利尿剂可控制的水肿;1,利尿剂不能控制的水肿);2)年龄为开始UCDA治疗时,胆红素、ALP、Alb及血小板计数为治疗1年后;ULN,正常值上限,LLN,正常值下限;3)基线幸存者功能即0.982(在5年时)、0.941(在10年时)、0.893(在15年时)。

    下载: 导出CSV
  • [1] LU M, ZHOU YR, HALLER IV, et al. Increasing prevalence of primary biliary cholangitis and reduced mortality with treatment[J]. Clin Gastroenterol Hepatol, 2018, 16( 8): 1342- 1350. e 1. DOI: 10.1016/j.cgh.2017.12.033.
    [2] ZENG N, DUAN WJ, 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] TRIVELLA J, JOHN BV, LEVY C. Primary biliary cholangitis: Epidemiology, prognosis, and treatment[J]. Hepatol Commun, 2023, 7( 6): e0179. DOI: 10.1097/HC9.0000000000000179.
    [4] TANAKA A, LEUNG PS, GERSHWIN ME. Environmental basis of primary biliary cholangitis[J]. Exp Biol Med(Maywood), 2018, 243( 2): 184- 189. DOI: 10.1177/1535370217748893.
    [5] HOURI I, HIRSCHFIELD GM. Primary biliary cholangitis: Pathophysiology[J]. Clin Liver Dis, 2024, 28( 1): 79- 92. DOI: 10.1016/j.cld.2023.06.006.
    [6] CHALIFOUX SL, KONYN PG, CHOI G, et al. Extrahepatic manifestations of primary biliary cholangitis[J]. Gut Liver, 2017, 11( 6): 771- 780. DOI: 10.5009/gnl16365.
    [7] EFE C, TORGUTALP M, HENRIKSSON I, et al. Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome[J]. J Gastroenterol Hepatol, 2021, 36( 4): 936- 942. DOI: 10.1111/jgh.15214.
    [8] 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. DOI: 10.1056/NEJM198301063080101.
    [9] DICKSON ER, GRAMBSCH PM, FLEMING TR, et al. Prognosis in primary biliary cirrhosis: Model for decision making[J]. Hepatology, 1989, 10( 1): 1- 7. DOI: 10.1002/hep.1840100102.
    [10] KIM WR, WIESNER RH, POTERUCHA JJ, et al. Adaptation of the Mayo primary biliary cirrhosis natural history model for application in liver transplant candidates[J]. Liver Transpl, 2000, 6( 4): 489- 494. DOI: 10.1053/jlts.2000.6503.
    [11] MIJIC M, SARIC I, DELIJA B, et al. Pretransplant evaluation and liver transplantation outcome in PBC patients[J]. Can J Gastroenterol Hepatol, 2022, 2022: 7831165. DOI: 10.1155/2022/7831165.
    [12] GOET JC, MURILLO PEREZ CF, HARMS MH, et al. A comparison of prognostic scores(mayo, UK-PBC, and GLOBE) in primary biliary cholangitis[J]. Am J Gastroenterol, 2021, 116( 7): 1514- 1522. DOI: 10.14309/ajg.0000000000001285.
    [13] FENG J, XU JM, FU HY, et al. Prognostic scores in primary biliary cholangitis patients with advanced disease[J]. World J Gastrointest Surg, 2023, 15( 8): 1774- 1783. DOI: 10.4240/wjgs.v15.i8.1774.
    [14] 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. DOI: 10.1016/0016-5085(85)90213-6.
    [15] 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.
    [16] 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. DOI: 10.1111/j.1478-3231.1999.tb00020.x.
    [17] 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.
    [18] 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.
    [19] 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. e5; quize 15. DOI: 10.1053/j.gastro.2014.08.029.
    [20] 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.
    [21] KUIPER EMM, 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. DOI: 10.1053/j.gastro.2009.01.003.
    [22] BALASUBRAMANIAM K, GRAMBSCH PM, WIESNER RH, et al. Diminished survival in asymptomatic primary biliary cirrhosis. A prospective study[J]. Gastroenterology, 1990, 98( 6): 1567- 1571. DOI: 10.1016/0016-5085(90)91091-J.
    [23] 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. DOI: 10.1007/s00535-009-0051-9.
    [24] AZEMOTO N, KUMAGI T, ABE M, et al. Biochemical response to ursodeoxycholic acid predicts long-term outcome in Japanese patients with primary biliary cirrhosis[J]. Hepatol Res, 2011, 41( 4): 310- 317. DOI: 10.1111/j.1872-034X.2011.00782.x.
    [25] 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. DOI: 10.1038/ajg.2010.216.
    [26] 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.
    [27] TABABI R, MRABET S, AKKARI I, et al. Prognostic scores in primary biliary cholangitis[J]. Future Sci OA, 2024, 10( 1): FSO975. DOI: 10.2144/fsoa-2023-0203.
    [28] TRIVEDI PJ, BRUNS T, CHEUNG A, et al. Optimising risk stratification in primary biliary cirrhosis: AST/platelet ratio index predicts outcome independent of ursodeoxycholic acid response[J]. J Hepatol, 2014, 60( 6): 1249- 1258. DOI: 10.1016/j.jhep.2014.01.029.
    [29] 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. DOI: 10.1200/JCO.2014.57.9151.
    [30] CHAN AWH, CHAN RCK, WONG GLH, et al. New simple prognostic score for primary biliary cirrhosis: Albumin-bilirubin score[J]. J Gastroenterol Hepatol, 2015, 30( 9): 1391- 1396. DOI: 10.1111/jgh.12938.
    [31] CHEN QL, ZHONG R, WANG Y, et al. The albumin-bilirubin score as a predictor of liver-related mortality in primary biliary cholangitis with compensated cirrhosis[J]. Dig Dis, 2023, 41( 6): 946- 956. DOI: 10.1159/000531557.
    [32] ITO T, ISHIGAMI M, MOROOKA H, et al. The albumin-bilirubin score as a predictor of outcomes in Japanese patients with PBC: An analysis using time-dependent ROC[J]. Sci Rep, 2020, 10( 1): 17812. DOI: 10.1038/s41598-020-74732-3.
    [33] 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. e 4. DOI: 10.1053/j.gastro.2015.07.061.
    [34] CARBONE M, HARMS MH, LAMMERS WJ, et al. Clinical application of the GLOBE and United Kingdom-primary biliary cholangitis risk scores in a trial cohort of patients with primary biliary cholangitis[J]. Hepatol Commun, 2018, 2( 6): 683- 692. DOI: 10.1002/hep4.1180.
    [35] SOHAL A, KOWDLEY KV. Primary biliary cholangitis: Promising emerging innovative therapies and their impact on GLOBE scores[J]. Hepat Med, 2023, 15: 63- 77. DOI: 10.2147/HMER.S361077.
    [36] CHEUNG AC, GULAMHUSEIN AF, JURAN BD, et al. External validation of the United Kingdom-primary biliary cholangitis risk scores of patients with primary biliary cholangitis treated with ursodeoxycholic acid[J]. Hepatol Commun, 2018, 2( 6): 676- 682. DOI: 10.1002/hep4.1186.
    [37] 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.
    [38] ALOMARI M, COVUT F, MOMANI L AL, et al. Evaluation of the United Kingdom-primary biliary cholangitis and global primary biliary cholangitis group prognostic models for primary biliary cholangitis patients treated with ursodeoxycholic acid in the U.S. population[J]. JGH Open, 2020, 4( 2): 132- 139. DOI: 10.1002/jgh3.12223.
    [39] CHEUNG KS, SETO WK, FUNG J, et al. Prognostic factors for transplant-free survival and validation of prognostic models in Chinese patients with primary biliary cholangitis receiving ursodeoxycholic acid[J]. Clin Transl Gastroenterol, 2017, 8( 6): e100. DOI: 10.1038/ctg.2017.23.
    [40] MARENCO-FLORES A, ROJAS AMARIS N, KAHAN T, et al. The external validation of GLOBE and UK-PBC risk scores for predicting ursodeoxycholic acid treatment response in a large U.S. cohort of primary biliary cholangitis patients[J]. J Clin Med, 2024, 13( 15): 4497. DOI: 10.3390/jcm13154497.
    [41] WANG R, MA JL, ZHANG FK, et al. Prediction of decompensation in patients with primary biliary cirrhosis[J]. Chin Hepatol, 2012, 17( 7): 460- 464.

    王蕊, 马佳丽, 张福奎, 等. 原发性胆汁性肝硬化发生失代偿的预测[J]. 肝脏, 2012, 17( 7): 460- 464.
  • 加载中
表(1)
计量
  • 文章访问数:  454
  • HTML全文浏览量:  162
  • PDF下载量:  39
  • 被引次数: 0
出版历程
  • 收稿日期:  2024-10-06
  • 录用日期:  2024-11-21
  • 出版日期:  2025-07-25
  • 分享
  • 用微信扫码二维码

    分享至好友和朋友圈

目录

    /

    返回文章
    返回