原发性胆汁性肝硬化患者自身抗体测定的意义

目的:探讨自身抗体测定对诊断原发性胆汁性肝硬化(PBC)的临床意义.方法:PBC患者52例和非PBC患者202例,其中包括自身免疫性肝炎(AIH)41例,原发性硬化型胆管炎(PSC)18例,乙型肝炎(HBV)89例,丙型肝炎(HCV)54例以及健康体检者40例,采用间接免疫荧光法(IIF)检测抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体(AMA)、抗心肌抗体(HRA)、抗骨骼肌抗体(ASA)、抗胃壁细胞抗体(PCA)、抗双链DNA(ds-DNA)抗体、抗肝肾微粒体抗体(LKM)、抗可溶性肝抗原(SLA)抗体和抗中性粒细胞胞质抗体(ANCA)等自身抗体,ELISA法检测抗髓过氧化酶抗体(MPO),并对其结果进行回顾性分析.结果:PBC患者中AMA阳性例数最高为46例(88.5%),ANA阳性率为71.2%(37/52);非PBC患者中阳性例数则分别为20例(9.9%)和51例(25.2%),两组比较,有非常显著性意义(P<0.01).AMA、SMA抗体检测在PBC与AIH患者中,均有非常显著性意义(P<0.01).但两组之间的ANA阳性率无显著性意义(P>0.05).PSC患者18例ANA阳性6例,AMA阳性7例均低于PBC患者.HBV,HCV感染患者检测ANA阳性率分别只有9.0%和22.2%;AMA阳性率也只有7.9%和3.7%,与PBC患者比较均有显著性差异(P<0.01).PBC患者及对照组检测ds-DNA,ANCA,LKM,SLA和MPO抗体结果显示PBC患者检测最高的ANCA阳性率为26.9%(14/52),其次是MPO阳性率为25.0%(13/52),与AIH比较,均有非常显著性意义(P<0.01).结论:血清自身抗体的检测对诊断、治疗和阻止原发性胆汁性肝硬化的发展有着十分重要作用.对提高PBC同其他疾病鉴别诊断和治疗有着非常重要的意义.     

自身抗体检测对肝病患者的鉴别诊断价值

目的探讨自身抗体检测在鉴别诊断自身免疫性肝病(autoimmune liver disease,ALD)与慢性乙型肝炎(chronic hepatitis B,CHB)患者中的价值。方法回顾性分析2011年5月-2014年8月经枣阳市第一人民医院确诊的ALD患者215例,其中自身免疫性肝炎(AIH)患者122例,原发性胆汁性肝硬化(PBC)患者93例,同时选取CHB患者453例,采用间接免疫荧光法和免疫印迹法检测ANA、AMA、SMA、抗SLA/LP、抗LKM-1、抗LC-1及AMA-M2。比较3组患者的ALT、AST、ALP、GGT水平。结果 AIH组、PBC组患者的ALT、AST、ALP、GGT含量高于CHB组,差异有统计学意义(P0.05);ALD患者ANA总阳性率为83.26%,高于CHB组(15.01%)(P0.05);AIH组ANA、AMA、SMA、抗SLA/LP、抗LKM-1、抗LC-1及AMA-M2阳性率分别为79.51%、14.75%、45.90%、16.39%、12.30%、6.565%、3.28%;PBC组ANA、AMA、SMA、抗SLA/LP、AMA-M2阳性率分别为88.17%、94.62%、11.83%、3.23%、94.62%;AIH组和PBC组ANA阳性检出率显著高于CHB组(P0.05)。AIH组和PBC组ANA滴度大于1∶320的比率显著高于CHB组(P0.05)。结论自身抗体检测是诊断ALD的必要条件,对ALD和CHB的鉴别诊断有重要临床意义。     

自身抗体检测在PBC和AIH诊断中的临床价值

目的探讨检测多种自身抗体在原发性胆汁性肝硬化（PBC）和自身免疫性肝炎（AIH）诊断中的临床价值。方法采用间接免疫荧光法检测抗核抗体（ANA）、采用免疫印迹法检测AMA—M2、抗Sp100、抗gp210、抗肝肾微粒体-1（LKM—I）、抗肝特异性胞质1型（LC-1）、抗可溶性肝抗原／肝胰抗原（抗SLA／LP）和抗Ro-52。结果在30例PBC患者,AMA—M2、抗Sp100、抗gp210、抗LKM-1、抗LC-1、抗SLA、抗R0—52和ANA阳性率分别为80．0％、13．3％、46．7％、0．0％、10．0％、6．7％、56．7％和66．7％,而在30例AIH患者,其阳件率分别为20．0％、10．0％、6．7％、10．0％、3．3％、10．0％、60％和90．0％;在PBC患者未检测出抗LKM—1,而其在A1H患者为11．11％。结论AMA—M2和抗gp210对PBC有较高的诊断价值,而LKM—1对AIH具有较高的诊断价值。     

原发性胆汁性肝硬化患者的免疫学特点分析

目的 分析原发性胆汁性肝硬化(PBC)出现的自身抗体等免疫学指标及其临床意义。 方法对3000例肝功能异常患者采用间接免疫荧光法检测抗核抗体(ANA)、抗线粒体抗体(AMA)、抗平滑肌抗体(SMA)和抗肝肾微粒体抗体(抗-LKM)等,并对ANA和AMA亚型及抗可溶性肝抗原/肝胰抗原(抗-SLA/LP)、LKM-1和抗肝特异性胞浆抗原型1抗体(抗-LC-1)等肝脏疾病相关的自身抗体进行了检测。结果 3000例肝病患者中,PBC 52例占1.7％。PBC患者的AMA和AMA-M2抗体均为阳性,52例PBC中,94.0％呈AMA高滴度(≥1:320)阳性,79.0％M2>200 RU/L,78.0％ANA阳性。ANA的主要荧光模式为细胞核膜型、细胞核点型和着丝点型。少见的荧光模式有抗干燥综合征A/B(SS-A/SS-B)、细胞核均质型、核仁型及颗粒型等。PBC患者免疫球蛋白M、碱性磷酸酶和γ-谷氨酰转肽酶高于乙型肝炎肝硬化患者;其白细胞介素(IL)-6、IL-10、肿瘤坏死因子α和干扰素γ水平高于正常人。5例表现为自身免疫性肝病重叠综合征,其中2例抗-SLA/LP阳性,提示PBC与自身免疫性肝炎(AIH)3型的重叠;1例抗-LKM-1阳性,提示PBC与AIH 2型的重叠;2例ANA阳性,且肝活体组织检查证实存在AIH和PBC的病理改变,提示为PBC与AIH 1型的重叠综合征。 结论 PBC在我国肝病患者中约占1％~2％。临床已出现典型症状者一     

不同肝病患者血清自身抗体谱阳性率调查

目的 调查不同肝病患者血清自身抗体谱阳性率的差异。方法 2019年3月～2022年3月我院诊治的自身免疫性肝病(ALD)患者92例【其中自身免疫性肝炎(AIH)51例,原发性胆汁性胆管炎(PBC)27例和原发性硬化性胆管炎(PSC)14例】,慢性乙型肝炎(CHB)61例,慢性丙型肝炎(CHC)15例,非酒精性脂肪性肝炎(NASH)患者34例,药物性肝损伤(DILI)患者27例健康体检者68例,使用免疫印迹法检测血清自身抗体。结果 ALD患者血清抗线粒体抗体M2亚型(AMA-M2)、抗可溶性肝抗原/抗肝胰抗原抗体(SLA/LP)、抗肝肾微粒体抗体Ⅰ型(LKM-1)、抗跨膜糖蛋白210(gp210)、抗肝细胞溶质抗原Ⅰ型(LC-1)、抗3E、抗早幼粒细胞性白血病(PML)和抗Ro-52阳性率分别为23.9%、13.0%、33.7%、19.6%、26.1%、33.7%、23.9%和55.4%,显著高于其他肝病组(P<0.05); AIH组患者血清抗SLA/LP、抗LKM-1和抗LC-1阳性率显著高于PBC组或PSC组(P<0.05),而PBC患者血清AMA-M2、抗gp210和抗...     

原发性胆汁性肝硬化患者自身抗体检测及其临床意义

目的 探讨血清自身抗体对原发性胆汁性肝硬化(PBC)的诊断及其临床意义.方法 对PBC患者采用间接免疫荧光、免疫印迹法检测抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体(AMA),并对AMAM2亚型及其抗可溶性肝抗原/肝胰抗原(SLA/Le)、抗肝肾微粒体Ⅰ型(LKM-1)和抗肝特异性胞浆Ⅰ型抗体(LC-1)等肝脏疾病相关的自身抗体进行检测.结果 PBC患者自身抗体以AMA和AMAM2亚型为主.其阳性率分别为96.5%和93.1%.患者的抗体滴度均大于1:100,其中有8例出现ANA和SMA,1例出现AMA和SLA/LP同时阳性,表现与Ⅰ型和Ⅲ型自身免疫性肝炎重叠,另有19例AMAM2阳性患者进行肝穿病理检查时,12例(63.7%)患者病理提示符合PBC诊断.结论 自身抗体对PBC有诊断意义,注重自身抗体的检测对明确自身免疫性肝病有重要的临床意义.     

原发性胆汁性肝硬化免疫学指标检测的临床意义

为研究原发性胆汁性肝硬化(PBC)免疫学指标测定的临床意义,选取32例确诊PBC的住院患者,采用ELISA法检测患者血清中的抗线粒体抗体M2亚型(AMA-M2);间接免疫荧光法检测抗线粒体抗体(AMA),抗核抗体(ANA),抗平滑肌抗体(SMA);斑点法检测抗肝肾微粒体抗体(LKM-1),抗肝溶质Ⅰ型抗原抗体(LC-1),抗可溶性肝抗原/肝胰抗原抗体(SLA/LP);免疫比浊法检测免疫球蛋白(IgG,IgA,IgM)和类风湿因子(RF)。同时观察AMA-M2与生化肝功指标的关系,比较PBC患者治疗前后AMA-M2的变化。结果显示诊断PBC意义最大的是AMA-M2,其阳性率最高达94%,其次是AMA和ANA,阳性率分别为88%和86%;AMA-M2值的高低与血清转氨酶、碱性磷酸酶和γ-谷氨酰转肽酶无关,熊去氧胆酸治疗前后AMA-M2变化不大。     

自身抗体联合检测在原发性胆汁性肝硬化诊断中的意义

目的探讨不同抗体联合检测在原发性胆汁性肝硬化(PBC)诊断中的意义。方法随机选择灵台县妇幼保健站2010年7月-2013年7月收治的肝病患者245例,根据患者所患疾病种类不同,将患者分为PBC组(n=162)、自身免疫性肝炎(AIH)组(n=42)和其他肝病患者(LDC)组(n=41)。采用ELISA法检测血清抗线粒体抗体M2亚型(AMA-M2)、抗SP100以及GP210抗体,采用Western Blot检测法检测血清中抗可溶性肝抗原(抗SLA)抗体,采用间接免疫荧光法检测血清抗线粒体抗体(抗AMA)。组间抗体阳性率的比较采用χ2检验和Fisher’s检验。结果 PBC患者的AMA、AMA-M2、抗GP210的阳性率分别是93.21%、87.65%、19.75%,高于AIH患者的19.05%、7.14%、9.52%,差异具有统计学意义(χ2=97.311,P=0.001;χ2=98.264,P=0.001;χ2=10.312,P=0.012)。PBC患者的AMA、AMA-M2及抗GP210的阳性率分别为93.21%、87.65%、19.75%,LDC患者的阳性率分别为9.76%、2.44%、7.32%,PBC患者明显高于LDC患者,差异具有统计学意义(χ2=142.745,P=0.003;χ2=112.574,P=0.002;χ2=15.217,P=0.042)。结论 AMA对PBC的诊断具有标志性意义,抗SP100对PBC的疾病进展具有提示作用,抗GP210对阴性PBC患者具有诊断作用。     

原发性胆汁性肝硬化的实验室指标分析

目的分析原发性胆汁性肝硬化(PBC)实验室指标,为临床诊治提供依据。方法选取2016年解放军第三〇二医院诊断为PBC的患者593例,对其自身抗体指标抗线粒体抗体(AMA)、抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体-M2(AMA-M2)、抗可溶性肝抗原/肝胰抗原抗体(SLP)、抗核孔复合物糖蛋白210抗体(gp210)、核点型靶抗原蛋白(Sp100)、早幼粒白血病蛋白(PML)以及生化免疫学指标谷氨酰转肽酶(GGT)、碱性磷酸酶(ALP)、免疫球蛋白A(IgA)、免疫球蛋白M(IgM)、免疫球蛋白G(IgG)进行检测并分析结果。结果PBC患者血清内ANA、AMA、SMA、AMA-M2、SLP/LP、gp210、Sp100的检出率显著高于慢性乙型肝炎组,差异均有统计学意义(P0.05)。ANA的主要核型是核膜型(225例,52.33%)以及核点型(90例,20.93%),滴度以中高滴度为主(≥1∶320),复合核型也有相当比例(113例,26.28%)。AMA以高滴度(≥1∶1 000)为主(470例,84.79%)。AMA-M2的滴度以高滴度(≥"3+")为主(358例,88.18%)。PBC组患者的GGT、ALP、IgM、IgG与慢性乙型肝炎组患者比较均显著升高,差异有统计学意义(P0.05),IgA差异无统计学意义(P0.05)。结论各型自身抗体对于PBC具有诊断意义,ALP、GGT、IgM不明原因升高也是PBC特殊指征,临床应该重视各种实验室指标,对于PBC患者早诊断早治疗,积极改善PBC患者的生存质量。     

肝功能异常患者中自身抗体及自身免疫性肝病的检测

目的 自身免疫性肝病临床流行病学调查,观察在肝功能异常患者中自身抗体检测的阳性率、自身免疫性肝病检出率及临床意义。方法 连续收集就诊病例中肝功能异常患者丙氨酸氨基转移酶(ALT)大于40 U/L血清511份,分别进行相关自身抗体(ANA)检测,并查阅临床资料。结果 511份血清检测出ANA阳性率为14.09％,抗平滑肌抗体(SMA)阳性率0,59％,抗线粒体抗体(AMA)阳性率2.94％,抗线粒体抗体亚型-丙酮酸脱氢酶复合物(AMA-M2)阳性率0.98％;ANA谱中的SS-A阳性率0.59％、SS-B阳性率0.20％、JO-1阳性率0.20％,dsDNA阳性率0.78％;未检出抗肝肾微粒体抗体(LKM-1)、可溶性肝抗原/肝胰抗原(SLA/LP)、抗肝细胞溶质抗原1型抗体(LC-1)及ANA谱中其他抗体。从ALT升高的患者中收集到的511份血清,可查到完整临床资料者共469例。原发性胆汁性肝硬化(PBC)及自身免疫性肝炎(AIH)患者检出率分别为1.06％及0.43％,未检出原发性硬化性胆管炎患者。自身抗体阳性患者77.78％诊断为病毒性肝炎及相关疾病。病毒性肝炎及相关疾病中自身抗体阳性率为18.29％。结论 高滴度(>1:320)自身抗体对自身免疫性肝病诊断有意义。PBC及AIH患者检出率近似丙型及戊型肝炎检出率,临床不能忽视,病毒性肝炎及相关疾病中可检测出自身抗体。     

Autoimmune liver serology：Current diagnostic and clinical challenges

Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases（AiLD）,namely autoimmune hepatitis types 1 and 2（AIH-1 and 2）,primary biliary cirrhosis（PBC）,and the sclerosing cholangitis variants in adults and children.AIH-1 is specified by anti-nuclear antibody（ANA） and smooth muscle antibody（SMA）.AIH-2 is specified by antibody to liver kidney microsomal antigen type-1（anti-LKM1） and anti-liver cytosol type 1（anti-LC1）.SMA,ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation.PBC is specified by antimitochondrial antibodies（AMA） reacting with enzymes of the 2-oxo-acid dehydrogenase complexes（chiefly pyruvate dehydrogenase complex E2 subunit） and disease-specific ANA mainly react-ing with nuclear pore gp210 and nuclear body sp100.Sclerosing cholangitis presents as at least two variants,first the classical primary sclerosing cholangitis（PSC） mostly affecting adult men wherein the only（and non-specific） reactivity is an atypical perinuclear antineutro-phil cytoplasmic antibody（p-ANCA）,also termed perinuclear anti-neutrophil nuclear antibodies（p-ANNA） and second the childhood disease called autoimmune sclerosing cholangitis（ASC） with serological features resembling those of type 1 AIH.Liver diagnostic serology is a fast-expanding area of investigation as new purified and recombinant autoantigens,and automatedtechnologies such as ELISAs and bead assays,become available to complement（or even compete with） traditional immunofluorescence procedures.We survey for the first time global trends in quality assurance impacting as it does on（1） manufacturers/purveyors of kits and reagents,（2） diagnostic service laboratories that fulfill clinicians＇ requirements,and（3） the end-user,the physician providing patient care,who must properly interpret test results in the overall clinical context.     

Autoimmune serology in liver disease: methodology and interpretation

Abstract   Accurate measurement of levels of autoantibodies in serum is critical for the diagnosis of autoimmune hepatitis. The major reactivities include anti-nuclear antibody (ANA), smooth muscle antibody (SMA), antibody to liver kidney microsomes type-1 (anti-LKM1); other relevant reactivities include antibodies to liver cytosol 1 (anti-LC1), soluble liver antigen (anti-SLA), and neutrophil cytoplasmic antigens (ANCA). In addition to the classical indirect immunofluorescence technique, automatic assays based on recombinant antigens are now available, which allow detection of antibodies not visible on immunofluorescence, like anti-SLA, and assist in the interpretation of at times problematic immunofluorescence patterns, like anti-LKM1 or anti-LC1.     

自身免疫性肝病109例的临床与病理特征分析

目的 分析比较自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)及AIH重叠综合征的临床特点、生化特征和组织学变化,以提高对自身免疫性肝病(AILD)的认识.方法 收集2004年1月-2008年6月肝穿刺病理学检查确诊的AILD患者共109例,其中AIH 27例、PBC 67例、PSC 4例、AIH-PSC重叠综合征1例和AIH-PBC重叠综合征10例,对患者的临床及实验室检查资料进行回顾性分析.结果 AILD患者多发于中年女性(73.3%,80/109),常见症状为黄疸、乏力、纳差和皮肤瘙痒.AIH患者的发病年龄高峰在50岁左右,肝功能检查结果显示为肝炎样异常,丙种球蛋白和免疫球蛋白G均明显高于正常值,62.9%的患者(17/27)抗核抗体(ANA)阳性.肝组织病理变化以界面性肝炎为主(77.7%),在重度患者则出现重度界面件肝炎、桥样坏死等.PBC患者主要表现为碱性磷酸酶、γ-谷氨酰转肽酶和胆红素明显升高,伴免疫球蛋白M升高,74.6%的患者(50/67)线粒体抗体(AMA)和(或)AMA-M2亚型阳性.所有PBC患者行肝脏病理学检查,早期(Ⅰ、Ⅱ)占28.3%,晚期(Ⅲ、Ⅳ)占71.7%,肝组织病理变化以小胆管减少甚至消失为主(62.6 0A).AIH-PBC重叠综合征患者的临床表现和肝组织病理学具有AlH和PBC的双重特征,其中有3例患者同时检测到ANA和AMA/AMA-M2阳性.结论 AILD在中国人中并非少见,其诊断需综合临床表现、生化、免疫指标和组织学变化.     

抗可溶性肝抗原/肝胰抗原抗体在自身免疫性肝炎诊断及分型中的意义

目的探讨抗可溶性肝抗原/肝胰抗原抗体（抗-SLA/LP）在AIH诊断及分型中的意义。方法对6000例采用间接免疫荧光法及免疫印迹法进行自身抗体检测的肝功能异常患者进行回顾性分析。结果6000例肝功异常患者中AIH患者84例,抗-SLA/LP阳性患者18例,占肝功能异常患者的0．3%。在18例抗-SLA/LP阳性患者中17例诊断为AIH,其中2例为AIH重叠原发性胆汁性肝硬化,1例诊断为慢性乙型肝炎。84例中AIH-Ⅰ型65例,AIH-Ⅱ型2例,AIH-Ⅲ型17例。抗-SLA/LP诊断AIH灵敏度为20．2%,特异度为99．7%,诊断AIH的阳性预测值为94．4%。17例AIH-Ⅲ型患者与65例AIH-Ⅰ型患者进行临床资料比较：AIH-Ⅲ型患者ANA滴度低于AIH-Ⅰ组,差异有统计学意义（P＜0．05）;抗-SLA/LP阳性组发病年龄在50岁以上者为58．8% （10/19）,而抗SLA/LP阴性组发病年龄在30～50岁者为52．2%（35/67）。但两组患者在性别构成、发病年龄、肝功能损害程度、凝血酶原活动度、IgG水平、肝硬化发生率及对激素治疗的反应方面的差异均无统计学意义。结论抗-SLA/LP对AIH的诊断有高度特异性。AIH-Ⅲ型患者除发病年龄偏高外,与Ⅰ型患者的临床特点比较,尚未发现差异有统计学意义。     

Autoantibodies and defined target autoantigens in autoimmune hepatitis: an overview

Autoimmune hepatitis (AIH) is a disease of unknown aetiology characterised by hypergammaglobulinaemia, non-organ and liver-related autoantibodies, association with HLA-DR3 or DR4 and a favourable response to immunosuppression. The current classification of AIH and the several autoantibodies/target autoantigens found in this disease are reported. The importance of these markers in the differential diagnosis and the study of pathogenesis of AIH is also given. AIH is subdivided into two major types: AIH type 1 (AIH-1) and AIH type 2 (AIH-2). AIH-1 is characterised by the detection of smooth muscle autoantibodies (SMA) and/or antinuclear antibodies (ANA). Antineutrophil cytoplasmic autoantibodies (ANCA), in most cases of perinuclear pattern (p-ANCA), by the indirect immunofluorescence assay, antibodies against the asialoglycoprotein receptor (anti-ASGP-R) and antibodies to soluble liver antigens or liver-pancreas (anti-SLA/LP) may be useful for the identification of individuals who are seronegative for ANA/SMA. AIH-2 is characterised by the presence of specific autoantibodies against liver and kidney microsomal antigens (anti-LKM type 1 or infrequently anti-LKM type 3) and/or autoantibodies against liver cytosol 1 antigen (anti-LC1). Anti-LKM-1 and anti-LKM-3 autoantibodies are also detected in some patients with chronic hepatitis C (HCV) and chronic hepatitis D (HDV). For these reasons, the distinction between AIH and chronic viral hepatitis is of particular importance. Cytochrome P450 2D6 (CYP2D6) is the major target autoantigen of anti-LKM-1 autoantibodies in both conditions (AIH-2 and HCV infection). Recent data have demonstrated the expression of CYP2D6 on the surface of hepatocytes, suggesting a pathogenetic role of anti-LKM-1 autoantibodies in liver injury. Family 1 of UDP-glycuronosyltransferases has been identified as the target autoantigen of anti-LKM-3. The molecular target of anti-SLA/LP autoantibodies has been identified recently as a 50 kDa protein with unknown structure and function. A liver-specific enzyme, the formiminotransferase cyclodeaminase, was identified as the target autoantigen of anti-LC1 autoantibodies. Anti-ASGP-R and anti-LC1 autoantibodies appear to correlate better with the severity of AIH and the response to treatment. The latter may suggest a pathogenic role of these autoantibodies in the hepatocellular damage in AIH. In general, however, autoantibodies should not be used to monitor treatment or to predict AIH activity or outcome. Finally, current knowledge concerning a specific form of AIH that may develop in some patients with a rare genetic syndrome, the autoimmune polyglandular syndrome type-1 (APS-1), is also discussed. Autoantibodies against liver microsomes (anti-LM) are the specific autoantibodies found in AIH as a disease component of APS-1. However, anti-LM autoantibodies have also been described in cases of dihydralazine-induced hepatitis. Cytochrome P450 1A2 has been identified as the target autoantigen of anti-LM autoantibodies in both disease entities.     

Overlap syndromes from pediatrics to adulthood

Abstract   Overlap syndromes are autoimmune conditions with mixed immunological, clinical and histological features. The most frequent overlaps are between primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH), and between AIH and sclerosing cholangitis (SC). True AIH/PBC overlap syndrome is rare and characterized by elevation of transaminases and immunoglobulin G (IgG), positive anti-smooth muscle antibodies and a liver biopsy showing interface hepatitis as well as changes typical of PBC. These patients respond to immunosuppressive treatment that must be given promptly, to avoid progression to liver failure. The so-called 'autoimmune cholangitis' defines a small group of patients with cholestatic and histological features of PBC but negative for anti-mitochondrial antibody (AMA) and positive for PBC-specific anti-nuclear antibody (ANA). The positivity for ANA in these patients is a consequence of the AMA negativity, since AMA masks ANA on immunofluorescence. These patients' clinical course and response to treatment resemble that of classical PBC. AIH/ASC overlap syndrome is characterised by elevated levels of IgG and circulating autoantibodies, including ANA, SMA and atypical perinuclear anti-neutrophil cytoplasmic antibody, in association with cholangiographic changes typical of SC. This condition affects in particular children and young adults and may represent the early stage of adult primary SC. The parenchymal liver inflammation responds satisfactorily to immunosuppression, while the bile duct damage may progress despite treatment.     

自身免疫性肝病的自身抗体检测及报告规范

自身抗体对自身免疫性肝病的诊断非常重要。抗线粒体抗体（AMA）及AMA-M2抗体是原发性胆汁性肝硬化（PBC）的诊断指标之一,具早期诊断作用,但与疗效和预后无相关性。PBC患者抗核抗体（ANA）有一定荧光模式,抗sp100和抗gp210对PBC有很高特异性。大部分自身免疫性肝炎（AIH）所出现的自身抗体缺乏疾病特异性,ANA和抗平滑肌抗体也可见于其他多种肝病。参照自身抗体将AIH分为两型,抗-SLA对AIH有极高特异性,阳性者病情多较重。自身抗体主要检测方法为间接免疫荧光法（IIF）、ELISA和免疫印迹法。ANA标准检测程序应为：IIF法对标本筛查,而后对必要者进行特异性ANA谱定量或半定量检测。自身抗体的室间质量评价是质量控制措施之一。     

Anti-liver cytosolic antigen type 1 (LC1) antibodies in childhood autoimmune liver disease

Antibodies to liver cytosol antigen type 1 (anti-LC1), which recognize a 60-kd peptide contained in the liver cytosolic fraction, have been reported to define a subset of autoimmune hepatitis (AIH) either negative for other autoantibodies or positive for anti-liver kidney microsomal antibody type 1 (LKM-1) and to be best detected in immunodiffusion. To analyze the prevalence of antiLC1 in childhood liver disease, we have tested the sera of 95 patients using immunoblot, indirect immunofluorescence, and immunodiffusion. Fifteen children had smooth muscle antibody (SMA) and/or anti-nuclear antibody (ANA)-positive AIH, 13 had anti-LKM-1-positive AIH, 14 had autoimmune sclerosing cholangitis (ASC) (all SMA and/or ANA positive), and 53 had non-autoimmune liver disease (10 had al-anti-trypsin deficiency [α1-ATD], 11 had Wilson's disease [wd], 14 had Alagille's syndrome, and 18 had chronic hepatitis B virus [HBV] infection). Twenty healthy children were studied as controls. Anti-LC1 positivity in immunodiffusion and strong reactivity in immunoblot were found in 4 LKM-1- and 2 SMA/ANA-positive patients with AIH and in 1 patient with ASC, but in none of the patients with other liver diseases nor in controls. A weak 60-kd band was detected by immunoblot in 6 more patients with AIH (2 were LKM-1- and 4 were SMA/ANA-positive) and 6 patients with ASC, all anti-LC1-negative by immunofluorescence and immunodiffusion. No distinct clinical features characterized the anti-LC1-positive patients. Our data show that, in pediatric age, LC1 reactivity, although associated with autoimmune liver disease, does not identify a distinct disease subset and that immunoblot is the most sensitive technique to detect anti-LC1.