中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Volume 35 Issue 3
Mar.  2019
Turn off MathJax
Article Contents

Clinical and pathological features of primary biliary cholangitis with negative anti-mitochondrial antibody

DOI: 10.3969/j.issn.1001-5256.2019.03.051
  • Published Date: 2019-03-20
  • Anti-mitochondrial antibody ( AMA) is a typical serum marker for primary biliary cholangitis ( PBC) , and the diagnosis ofAMA-negative PBC may easily be neglected in clinical practice. This article analyzes the differences in epidemiology, clinical and patho-logical features, and treatment outcome between AMA-negative and AMA-positive PBC and points out that there are significant differencesbetween them in the symptom of pruritus, immunoglobulin M, and severity of bile duct injury. For patients with a clinical diagnosis of AMA-negative PBC, immunofluorescence assay combined with immunological detection should be performed to exclude the false-negative resultof AMA, and a confirmed diagnosis can be made with reference to the manifestation of cholestasis, positive results of anti-sp100, anti-gp210, and anti-ANA antibodies, and typical hyperactive cholangitis based on pathological examination. Differential diagnosis of this dis-ease with other types of bile duct injury or absence of bile duct should be taken seriously in clinical practice.

     

  • loading
  • [1] Chinese Society of Hepatology, Chinese Medical Association;Chinese Society of Gastroenterology, Chinese Medical Associ-ation; Chinese Society of Infectious Diseases, Chinese Medi-cal Association. Consensus on the diagnosis and managementof primary biliary cirrhosis (cholangitis) (2015) [J]. J ClinHepatol, 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.
    [2] BOONSTRA K, BEUERS U, PONSIOEN CY. Epidemiology ofprimary sclerosing cholangitis and primary biliary cirrhosis:Asystematic review[J]. J Hepatol, 2012, 56 (5) :1181-1188.
    [3] European Association for the Study of the Liver. EASL ClinicalPractice Guidelines:The diagnosis and management of pa-tients with primary biliary cholangitis[J]. J Hepatol, 2017, 67 (1) :145-172.
    [4] LUO LL, ZHOU L, WANG BM. Clinical, biochemical and patho-logical features of anti-mitochondrial antibody-negative primarybiliary cirrhosis[J]. Shandong Med J, 2014, 54 (7) :5-8. (inChinese) 罗莉丽, 周璐, 王邦茂.抗线粒体抗体阴性原发性胆汁性肝硬化患者的临床、生化和病理学特点[J].山东医药, 2014, 54 (7) :5-8.
    [5] JULIUSSON G, IMAM M, BJORNSSON ES, et al. Long-termoutcomes in antimitochondrial antibody negative primary biliarycirrhosis[J]. Scand J Gastroenterol, 2016, 51 (6) :745-752.
    [6] LIU WY, XIAO L, YIN H, et al. Clinical and pathological fea-tures of primary biliary cirrhosis in anti-mitochondriaantibody/anti-mitochondrial antibody M2 subtype negative patients[J]. World Chin J Dig, 2013, 21 (34) :3799-3805. (in Chi-nese) 刘卫英, 肖琳, 银皓, 等. AMA/AMA-M2阴性原发性胆汁性肝硬化患者的临床及病理特征[J].世界华人消化杂志, 2013, 21 (34) :3799-3805.
    [7] RASZEJA-WYSZOMIRSKA J, WUNSCH E, KRAWCZYK M, et al. Assessment of health related quality of life in polish pa-tients with primary biliary cirrhosis[J]. Clin Res Hepatol Gas-troenterol, 2016, 40 (4) :471-479.
    [8] YANG JH, ZHANG ZJ. Autoimmune liver disease:Clinical andpathology[M]. Beijing:People's Health Publishing House, 2011:138. (in Chinese) 杨晋辉, 章宗籍.自身免疫性肝病—临床与病理[M].北京:人民卫生出版社, 2011:138.
    [9] LAMMERS WJ, BUUREN HR, HIRSCHFIELD GM, et al. Lev-els of alkaline phosphatase and bilirubin are surrogate endpoints of outcomes of patients with primary biliary cirrhosis:Aninternational follow-up study[J]. Gastroenterology, 2014, 147 (6) :1338-1349, e15.
    [10] HU W. Evaluation on autoantibodies and immune parametersin diagnosis of primary biliary cirrhosis[J]. Guangxi Med J, 2014, 36 (11) :1565-1567. (in Chinese) 胡伟.自身抗体和免疫指标在原发性胆汁性肝硬化诊断中的价值[J].广西医学, 2014, 36 (11) :1565-1567.
    [11] SAVERINO D, PESCE G, ANTOLA P, et al. High levels of sol-uble CTLA-4 are present in anti-mitochondrial antibody pos-itive, but not in antibody negative patients with primary biliarycirrhosis[J]. PLo S One, 2014, 9 (11) :e112509.
    [12] WANG Y, LIU XH, ZHANG XZ, et al. The analysis and com-parison of anti-mitochondrial antibody detected by indirectimmunofluorescence and enzyme linked immunosorbent assay[J]. J China-Japan Friendship Hosp, 2015, 29 (3) :154-157. (in Chinese) 王艳, 刘晓辉, 张学智, 等.间接免疫荧光法和酶联免疫吸附法检测抗线粒体抗体的分析与比较[J].中日友好医院学报, 2015, 29 (3) :154-157.
    [13] CHEN C, ZHANG W. The positive incidence and diagnostic sig-nificance of AMA-M2 in PBC of abnomal ALT[J]. Chin J Gastro-enterol Hepatol, 2017, 26 (9) :1022-1025. (in Chinese) 陈辰, 张玮.血清AMA-M2抗体在ALT异常人群的检出率和诊断PBC的价值[J].胃肠病学和肝病学杂志, 2017, 26 (9) :1022-1025.
    [14] European Association for the Study of the Liver. EASL ClinicalPractice Guidelines:The diagnosis and management of pa-tients with primary biliary cholangitis[J]. J Hepatol, 2017, 67 (1) :145-172.
    [15] LIU R, LU LG. Advances of the researth of the value of antimi-tochondrial antibody in the diagnosis of primary biliary cirrhosis[J]. J Prac Hepatol, 2014, 17 (2) :218-221. (in Chinese) 刘然, 陆伦根.抗线粒体抗体对原发性胆汁性肝硬化诊断价值研究进展[J].实用肝脏病杂志, 2014, 17 (2) :218-221.
    [16] LUETTIG B, BOEKER KH, SCHOESSLER W, et al. The anti-nuclear autoantibodies Sp100 and gp210 persist after ortho-topic liver transplantation in patients with primary biliary cirrho-sis[J]. J Hepatol, 1998, 28 (5) :824-828.
    [17] BOWLUS CL, GERSHWIN ME. The diagnosis of primary biliarycirrhosis[J]. Autoimmunity Rev, 2014, 13 (4-5) :441-444.
    [18] Special Committee of Autoantibody Detection, Branch of Rheuma-tology&Immunology Doctors, Chinese Medical Doctor Associa-tion. Expert consensus on the clinical application of anti-nuclearantibody detection[J]. Chin J Lab Med, 2018, 41 (4) :275-280. (in Chinese) 中国医师协会风湿免疫科医师分会自身抗体检测专业委员会.抗核抗体检测的临床应用专家共识[J].中华检验医学杂志, 2018, 41 (4) :275-280.
    [19] HU SL, ZHAO FR, HU Q, et al. Meta-analysis assessmentof GP210 and SP100 for the diagnosis of primary biliary cirrho-sis[J]. PLo S One, 2014, 9 (7) :e101916.
    [20] YANG F, YANG Y, WANG Q, et al. The risk predictive values ofUK-PBC and GLOBE scoring system in Chinese patients withprimary biliary cholangitis:The additional effect of anti-gp210[J]. Aliment Pharmacol Ther, 2017, 45 (5) :733-743.
    [21] YANG Z, LIANG Y, LIN F, et al. Implication of increased ser-um stromal cell-derived factor-1 for primary biliary cholangi-tis[J]. Int Immunopharmacol, 2018, 56:285-290.
    [22] NORMAN GL, YANG CY, OSTENDORFF HP, et al. Anti-kelch-like 12 and anti-hexokinase 1:Novel autoantibodies in primarybiliary cirrhosis[J]. Liver Int, 2015, 35 (2) :642-651.
    [23] LISO F, MATINATO C, RONCHI M, et al. The diagnostic ac-curacy of biomarkers for diagnosis of primary biliary cholangitis (PBC) in anti-mitochondrial antibody (AMA) -negative PBCpatients:A review of literature[J]. Clin Chem Lab Med, 2017, 56 (1) :25-31.
    [24] TAN YG, YAN HP, GAO ZH. Research progress in role of Bcell immunity in primary biliary cirrhosis[J]. J Clin Hepatol, 2014, 30 (5) :400-404. (in Chinese) 谭延国, 闫惠平, 高祖华.原发性胆汁性肝硬化中B细胞免疫的研究进展[J].临床肝胆病杂志, 2014, 30 (5) :400-404.
    [25] JIN Q, MORITOKI Y, LLEO A, et al. Comparative analysis ofportal cell infiltrates in antimitochondrial autoantibody-positiveversus antimitochondrial autoantibody-negative primary biliarycirrhosis[J]. Hepatology, 2012, 55 (5) :1495-1506.
    [26] FANG JY, ZHU ZP, LI K, et al. Expression of PDC-E2 anti-body in autoimmune liver diseases[J]. Chin Hepatol, 2017, 22 (1) :34-37. (in Chinese) 方静怡, 朱正鹏, 李凯, 等. PDC-E2抗体在自身免疫性肝病的表达研究[J].肝脏, 2017, 22 (1) :34-37.
    [27] WANG WB. Aberrant B cell immune response in primary biliarycholangitis[D]. Guangzhou:Southern Medical University, 2017. (in Chinese) 王卫彬. B细胞免疫紊乱在原发性胆汁性胆管炎中的研究[D].广州:南方医科大学, 2017.
    [28] TAKAHASHI T, MIURA T, NAKAMURA J, et al. Plasma cellsand the chronic nonsuppurative destructive cholangitis of pri-mary biliary cirrhosis[J]. Hepatology, 2012, 55 (3) :846-855.
    [29] YOU Z, WANG Q, BIAN Z, et al. The immunopathology of liv-er granulomas in primary biliary cirrhosis[J]. J Autoimmun, 2012, 39 (3) :216-221.
    [30] ZHANG LN, SHI TY, SHI XH, et al. Early biochemical responseto ursodeoxycholic acid and long-term prognosis of primary bili-ary cirrhosis:Results of a 14-year cohort study[J]. Hepatology, 2013, 58 (1) :264-272.
    [31] WANG LH, FU GS, YUAN J. Clinical observation of primarybiliary cirrhosis patients with positive of negative AMA-M2and follow-up after ursodeoxycholic acid treatment[J]. ChinJ Clin Res, 2011, 24 (5) :391-392. (in Chinese) 王立红, 付广双, 袁静. AMA-M2阳性和阴性的PBC患者临床观察及UDCA治疗随访[J].中国临床研究, 2011, 24 (5) :391-392.
    [32] CARBONE M, MELLS GF, PELLS G, et al. Sex and age aredeterminants of the clinical phenotype of primary biliary cirrho-sis and response to ursodeoxycholic acid[J]. Gastroenterolo-gy, 2013, 144 (3) :560-569, e13-e14.
    [34] VIEIRA BJ, VIONNET J, SCIARRA A, et al. Primary biliary cholangi-tis:An update[J]. Rev Med Suisse, 2018, 14 (616) :1489-1494.
    [35] NI P, FAN XL, WEN MY, et al. New advances in the treatmentof refractory primary biliary cholangitis[J]. J Clin Hepatol, 2017, 33 (11) :2230-2234. (in Chinese) 倪萍, 凡小丽, 文茂瑶, 等.难治性原发性胆汁性胆管炎的治疗新进展[J].临床肝胆病杂志, 2017, 33 (11) :2230-2234.
  • 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 (1917) PDF downloads(341) Cited by()
    Proportional views
    Related

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return