自身免疫性肝病临床诊治研究进展*

doi:10.3969/j.issn.1672-5069.2016.03.030

摘要/Abstract

摘要： 自身免疫性肝病（AILD）的检出和诊断率逐年提高，并已成为肝脏炎症性疾病的研究热点。本文归纳了近年来AILD诊疗的最新进展，重点介绍较为确切的诊断方法、难治性AILD替代治疗方案的选择、原发性硬化性胆管炎(PSC)与IgG4相关性胆管炎(IAC)的鉴别诊断，为临床医生对该类疾病的诊治提供帮助。

关键词: 自身免疫性肝病, 自身免疫性肝炎, 原发性硬化性胆管炎, IgG4相关性胆管炎

Abstract: With the increasing diagnosis of autoimmune liver diseases（AILD），the hot spot is on the study of diagnostic methods，differential diagnosis and managements. In this paper，we especially put our focus on the accurate diagnosis，the replacement therapy about the intractable AILD and the differentiation between primary sclerosing cholangitis and immunoglobulin G4 associated cholangitis.

Key words: Autoimmune liver diseases, Autoimmune hepatitis, Primary sclerosing cholangitis, Immunoglobulin G4 associated cholangitis

艾月梅，严玉娟，邢益平. 自身免疫性肝病临床诊治研究进展*[J]. 实用肝脏病杂志, 2016, 19(3): 360-364.

Ai Yuemei,Yan Yujuan,Xing Yiping. Autoimmune liver diseases: the mist really clears?[J]. JOURNAL OF PRACTICAL HEPATOLOGY, 2016, 19(3): 360-364.

参考文献

[1] Beuers U，Gershwin ME，Gish RG，et al. Changing nomenclature for PBC:from 'cirrhosis' to 'cholangitis'. Gut，2015，64（11）:1671-1672.
[2] Gronbaek L，Vilstrup H，Jepsen P，et al. Autoimmune hepatitis in Denmark:Incidence，prevalence，prognosis，and causes of death. A nationwide registry-based cohort study. J Hepatol，2013，60:612-617.
[3] 邹正升，李宝森，黄昂，等.近10年4种常见的非感染性肝病患者临床特征及变化趋势分析.实用肝脏病杂志，2014，17（5）：475-478.
[4] Hennes EM，Zeniya M，Czaja AJ，et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology，2008，48（1）:169-176.
[5] Czaja AJ. Performance parameters of the diagnostic scoring systems for autoimmune hepatitis. Hepatology，2008，48（5）:1540-1548.
[6] Qiu D，Wang Q，Wang H，et al. Validation of the simplified criteria for diagnosis of autoimmune hepatitis in Chinese patients. J Hepatol，2011，54（2）:340-347．
[7] de Boer YS，van Nieuwkerk CM，Witte BI，et a1. Assessment of the histopathological key features in autoimmune hepatitis. Histopathology，2015，66（3）:351-362.
[8] Manns MP，Czaja AJ，Gorham JD，et al. Diagnosis and management of autoimmune hepatitis. Hepatology，2010，51（6）: 2193-2213.
[9] 张敏，韩涛，聂彩云，等. 自身免疫性肝病患者血清IgG4水平分析. 实用肝脏病杂志，2014，17（4）：396-399.
[10] Manns MP，Woynarowski M，Kreisel W，et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology，2010，139（4）:1198-1206.
[11] Than NN，Wiegard C，Weiler-Normann C，et al. Long-term follow-up of patients with difficult to treat type 1 autoimmune hepatitis on Tacrolimus therapy. Scand J Gastroenterol，2016，51（3）:329-336.
[12] Manns MP，Lohse AW，Vergani D. Autoimmune hepatitis update-2015. J Hepatol，2015，62（1 Suppl）:S100-S111.
[13] Lindor KD，Gershwin ME，Poupon R，et al. Primary biliary cirrhosis. Hepatology，2009，50（1）:291-308.
[14] Van Norstrand MD，Malinchoc M，Lindor KD，et al. Quantitative measurement of autoantibodies to recombinant mitochondrial antigens in patients with primary biliary cirrhosis: relationship of levels of autoantibodies to disease progression. Hepatology，1997，25（1）:6-11.
[15] Tanaka A，Miyakawa H，Luketic VA，et al. The diagnostic value of anti-mitochondrial antibodies，especially in primary biliary cirrhosis. Cell Mol Biol （Noisy-le-grand），2002，48（3）:295-299.
[16] 王绮夏，沈镭，陈晓宇，等. 抗线粒体抗体阴性原发性胆汁性肝硬化患者的临床及病理学特点.中华肝脏病杂志，2011，19（5）:340-344.
[17] 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.世界华人消化杂志，2013，21（34）: 3799-3805.
[18] Beuers U，Lindor KD. A major step towards effective treatment evaluation in primary biliary of cirrhosis. J Hepatol，2011，55（6）:1178-1180.
[19] Corpechot C，Chazouillères O，Poupon R. Early primary biliary cirrhosis:biochemical response to treatment and prediction of long-term outcome. J Hepatol，2011，55（6）:1361-1367.
[20] Hirschfield GM，Mason A，Luketic V，et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Gastroenterology，2015，148（4）:751-761.
[21] 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. Hepatology，2012，55（2）:512-521.
[22] Lombard M，Portmann B，Neuberger J，et al. Cyclosporin A treatment in primary biliary cirrhosis:results of a long-term placebo controlled trial. Gastroenterology，1993，104（2）:519-526.
[23] Jones EA，ten Kate FJ，ter Borg F，et al. Combination therapy with mycophenolate mofetil and ursodeoxycholic acid for primary biliary cirrhosis. Eur J Gastroenterol Hepatol，1999，11:1165-1169.
[24] 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. Hepatology，2000，31:318-323.
[25] Mazzella G，Fusaroli P，Pezzoli A，et al. Methylprednisolone administration in primary biliary cirrhosis increases cholic acid turnover，synthesis，and deoxycholate concentration in bile. Dig Dis Sci，1999，44:2478-2483.
[26] Lindor KD，Kowdley KV，Harrison ME，et al. ACG Clinical Guideline:Primary sclerosing cholangitis. Am J Gastroenterol， 2015，110（5）：646-659.
[27] Lankisch TO，Metzger J，Negm AA，et al. Bile proteomic profiles differentiate cholangiocarcinoma from primary sclerosing cholangitis and choledocholithiasis. Hepatology，2011，53（3）:875-884.
[28] Metzger J，Negm AA，Plentz RR，et al. Urine proteomic analysis differentiates cholangiocarcinoma from primary sclerosing cholangitis and other benign biliary disorders. Gut，2013，62（1）:122-130.
[29] Olsson R，Boberg KM，de Muckadell OS，et al. High-dose ursodeoxycholic acid in primary sclerosing cholangitis:a 5-year multicenter，randomized，controlled study. Gastroenterology，2005，129（5）:1464-1472.
[30] Wunsch E，Trottier J，Milkiewicz M，et al. Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis. Hepatology，2014，60（3）:931-940.
[31] Frkkil M，Karvonen AL，Nurmi H，et al. Metronidazole and ursodeoxycholic acid for primary sclerosing cholangitis:a randomized placebo-controlled trial. Hepatology，2004，40（6）:1379-1386.
[32] Zhu GQ，Shi KQ，Huang GQ，et al. A network meta-analysis of the efficacy and side effects of udca-based therapies for primary sclerosing cholangitis. Oncotarget，2015，6（29）:26757-2669.
[33] Stiehl A，Rudolph G，Kl?ters-Plachky P，et al. Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid:outcome after endoscopic treatment. J Hepatol，2002，36（2）:151-156.
[34] Enns R，Eloubeidi MA，Mergener K，et al. Predictors of successful clinical and laboratory outcomes in patients with primary sclerosing cholangitis undergoing endoscopic retrograde cholangiopancreatography. Can J Gastroenterol，2003，17（4）:243-248.
[35] Rubel LR，Seeff LB，Patel V. Primary biliary cirrhosis-primary sclerosing cholangitis overlap syndrome. Arch Pathol Lab Med， 1984，108（5）:360-361.
[36] Burak KW，Urbanski SJ，Swain MG. A case of coexisting primary biliary cirrhosis and primary sclerosing cholangitis:a new overlap of autoimmune liver diseases. Dig Dis Sci，2001，46（9）:2043-2047.
[37] Chazouillères O1，Wendum D，Serfaty L，et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome:clinical features and response to therapy. Hepatology，1998，28（2）:296-301.
[38] Boberg KM，Chapman RW，Hirschfield GM，et al. Overlap syndromes:the International Autoimmune Hepatitis Group（IAIHG） position statement on a controversial issue. J Hepatol，2011，54（2）:374-385.
[39] EASL Clinical Practice Guidelines. Management of cholestatic liver diseases. J Hepatol，2009，51（2）:237-267.
[40] 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. Clin Gastroenterol Hepatol，2014，12（5）:863-869.
[41] Lüth S，Kanzler S，Frenzel C，et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J Clin Gastroenterol，2009，43（1）:75-80.
[42] Yoshida K，Toki F，Takeuchi T，et al. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci，1995，40（7）:1561-1568.
[43] Kamisawa T，Funata N，Hayashi Y，et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol，2003，38（10）:982-984.
[44] Bjrnsson E，Chari ST，Smyrk TC，et al. Immunoglobulin G4 associated cholangitis:description of an emerging clinical entity based on review of the literature. Hepatology，2007，45（6）:1547-1554.
[45] Ghazale A，Chari ST，Zhang L，Immunoglobulin G4-associated cholangitis:clinical profile and response to therapy. Gastroenterology，2008，134（3）:706-715.
[46] Ohara H，Okazaki K，Tsubouchi H，et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci，2012，19（5）:536-542.
[47] Tokala A，Khalili K，Menezes R，et al. Comparative MRI analysis of morphologic patterns of bile duct disease in IgG4-related systemic disease versus primary sclerosing cholangitis. AJR，2014，202:536-543.
[48] Gardner CS，Bashir MR，Marin D，et al. Diagnostic performance of imaging criteria for distinguishing autoimmune cholangiopathy from primary sclerosing cholangitis and bile duct malignancy. Abdom Imaging，2015，40（8）:3052-3061.
[49] Buijs J，van Heerde MJ，Rauws EA，et al. Comparable efficacy of low- versus high-dose induction corticosteroid treatment in autoimmune pancreatitis. Pancreas，2014，43:261-267.