Serial changes in enzyme inhibitory antibody to pyruvate dehydrogenase complex during the course of primary biliary cirrhosis

To assess the usefulness of enzyme inhibition assay for the diagnosis of primary biliary cirrhosis (PBC), we determined the serial changes in enzymatic inhibitory antibody to pyruvate dehydrogenase complex (PDC) in patients with PBC, and compared the results to those of immunofluorescence and immunoblotting. Forty-nine sera from 19 patients with PBC who were followed-up for at least 16 months were tested for antimitochondrial antibodies (AMA) by indirect immunofluorescence, immunoblotting on bovine heart mitochondria, and enzyme inhibition assay using commercially available TRACE Enzymatic Mitochondrial Antibody (M2) Assay (EMA) kit. Of the 49 sera, 39 (80%), 35 (71%), 38 (78%), 31 (63%), and 36 (73%) were positive for AMA by immunofluorescence, for immunoglobulin G (IgG), IgM, and IgA class antibody against E2 subunit of PDC (PDC-E2) by immunoblotting, and for enzymatic inhibitory antibody to PDC by EMA, respectively. AMA titers determined by immunofluorescence did not change in 9 patients (47%), increased in 4 (21%), decreased in 3 (16%), and fluctuated in 3 (16%) during follow-up. The number of anti-M2 bands by immunoblotting did not change in 9 (47%), increased in 6 (32%), decreased in 2 (11%), and fluctuated in 2 (11%). Units of PDC activity by EMA did not change markedly in 16 (84%), increased in 2 (11%), and fluctuated in 1 (5%). Positive EMA results were common in cases with high levels of serum alkaline phosphatase and IgM, and the units of PDC activity by EMA correlated significantly and inversely with AMA titers by immunofluorescence, and serum reactivity to PDC-E2 by immunoblotting, respectively. There was no correlation between serial changes in biochemical data and units of PDC activity by EMA. In three patients who showed a decrease in AMA titers, AMA titers correlated more with EMA results than immunoblotting. Moreover, in a patient with fluctuating AMA titers, the units of PDC activity by EMA paralleled AMA titers. Our results suggest that EMA is useful for the diagnosis of AMA-positive PBC, and also could be used for monitoring the disease course in PBC.     

Peripheral blood T-cell responses to pyruvate dehydrogenase complex in primary biliary cirrhosis: role of antigen-presenting dendritic cells

Patients with primary biliary cirrhosis (PBC) are usually characterized by the presence of antibody to pyruvate dehydrogenase complex (PDC) in the sera and PDC-specific T cells in the liver. However, most of the patients with PBC do not show peripheral blood T cells response to PDC. In this study, we re-evaluated the peripheral blood T cell responses to PDC in PBC using antigen-presenting dendritic cells (DCs). Twenty-four patients with PBC (AMA-positive: 16; AMA-negative: 8) and 13 normal controls were enrolled in the study. Peripheral blood mononuclear cells (PBMC) and highly enriched populations of T cells were stimulated with either only PDC or DCs plus PDC or PDC-pulsed DC plus PDC. Antibodies to different components of PDC were estimated by an immunoblotting technique. PBMC from only one out of ten AMA-positive PBC patients proliferated when cultured with only PDC. However, peripheral blood T cells from ten out of ten AMA-positive PBC patients and three out of ten AMA-negative PBC patients, but none of the five normal controls showed PDC-specific proliferation when cultured with PDC-pulsed DCs. Two of these three AMA-negative PBC patients, although negative for AMA, were positive for antibodies to other components of PDC. PDC-specific T cells are present in the peripheral blood from most of the patients with PBC. This is the first report on the effectiveness of antigen-pulsed DCs for the elucidation of autoantigen-specific immune response in human autoimmune diseases.     

M2抗体人源靶抗原三联体的克隆表达及其在原发性胆汁性肝硬化中的应用

目的 设计克隆表达抗原蛋白三联体BPO，利用纯化的重组BPO，建立原发性胆汁性肝硬化(primary biliary cirrhosis，PBC)特异性免疫学诊断方法。方法 利用基因工程方法克隆表达M2抗原及其三联体BP0，并加以鉴定。纯化BPO，建立ELISA法。应用ELISA法检测17例PBC患血清M2抗体，以167例非PBC患和1225例健康人作对照。结果 17例临床诊断为PBC的患，M2抗体均为阳性，而对照组M2抗体均为阴性。结论 本法检测M2抗体有较好的敏感性及特异性，为PBC的临床诊断提供了有效手段。     

用ELISA检测抗丙酮酸脱氢酶复合物E2和E3结合蛋白自身抗体

目的　用重组丙酮酸脱氢酶复合物E2亚单位和E3结合蛋白 (PDC E2 ,PDC E3BP)建立筛查原发性胆汁性肝硬化 (PBC)患者相应自身抗体的ELISA。方法　用本室制备的重组PDC E2和PDC E3BP蛋白包被酶联反应板 ,检测PBC患者、正常人及其他肝病患者血清。结果　组建成筛查PBC患者血清中自身抗体的试剂盒 ,能够准确地检测PBC患者血清中的自身抗体。结论　建立的试剂盒能用于PBC患者血清中丙酮酸脱氢酶复合物自身抗体的检测。     

自身免疫性肝病及其自身抗体谱分析

目的探讨重庆地区不同类型自身免疫性肝病患者血清中自身抗体谱的特征及其临床应用价值。方法 743例肝病送检样本中81例确诊为自身免疫性肝病,将其分为3组:(1)自身免疫性肝炎(AIH)38例;(2)原发性胆汁性肝硬化(PBC)30例;(3)重叠综合征13例。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)、抗肝肾微粒抗体(LKM)、抗可溶性肝抗原(SLA)等,用酶联免疫吸附试验(ELISA)定量测定抗线粒体M2亚型。结果 743例中诊断为AIH、PBC和重叠综合征分别为送检标本的5.11%、4.04%和1.75%,总计10.9%。AIH患者ANA阳性率为78.9%,AMA及M2亚型阳性率为18.4%,SMA阳性率为7.89%;PBC患者ANA阳性率为73.3%;AMA和M2阳性率为26.7%;重叠综合征患者ANA及AMA阳性率为100%。结论自身免疫性肝病抗体谱检测有助于自身免疫性肝病诊断,非病毒性肝炎诊断时应考虑自身免疫性肝病。     

Detection of autoantibodies against M2, LKM-1, and SLA in liver diseases by standardized uniform ELISA-techniques

Antibodies directed against soluble liver antigen (SLA), liver kidney microsomal antigen (LKM-1-AG), and antimitochondrial antigen M2 (M2-AMA) are critical serological markers for the differential diagnosis of autoimmune chronic active hepatitis (AI-CAH) and primary biliary cirrhosis (PBC). The exact diagnosis of autoimmune hepatitis and PBC is of great clinical relevance, as it leads to different therapeutic strategies. In the present work, a simple and reliable ELISA test system is described, which applies the same test principle for the detection of three different species of autoantibodies important for the diagnosis of chronic liver disease. The ELISA assays are based on a competitive inhibition of binding of positive standard antibodies by patients sera containing antibodies of unknown specificity. The purified immunoglobulins of clinically and serologically clearly defined patients with SLA or LKM-1 positive AI-CAH and with M2 positive PBC were used as coating- and detection antibodies in the ELISAs. From homogenized rat liver the fractionated 100,000g supernatant was employed for the SLA ELISA, the microsomal preparation served as antigen for the LKM-1 ELISA and the mitochondrial preparation was used for the M2 ELISA. In 1,500 sera of patients with the differential diagnosis of a hepatobiliary disease, 17 gave a positive signal in the SLA ELISA, 12 in the LKM-1-ELISA, and 72 in the M2-ELISA. The results of the ELISAs were compared with Western blotting and immunofluorescence staining pattern on cryostat sections and Hep2 cells. The antibody profiles of several patients are described in detail. © 1994 Wiley-Liss, Inc.     

血清抗线粒体抗体M_2亚型检测在原发性胆汁性肝硬化临床诊断中的价值

目的:探讨抗线粒体抗体(AMA)-M2亚型检测在原发性胆汁性肝硬化(primary billiary cirrhosis,PBC)诊断中的价值。方法:应用酶联免疫吸附试验,检测20名健康对照者、139例其他非PBC疾病患者和42例PBC患者血清中的AMA-M2水平,并采用矩阵图决策法分析血清AMA-M2检测对PBC的诊断效率。结果:健康对照者平均血清AMA-M2水平为(16.51±1.38)RU/mL,非PBC患者的平均血清AMA-M2水平为(24.33±7.29)RU/mL,两者比较差异无统计学意义(P>0.05)。PBC患者的平均AMA-M2水平为(211.51±126.68)RU/mL,与健康对照者比较,差异有统计学意义(P<0.01)。AMA-M2检测对PBC诊断的灵敏度为95.2%,特异度为97.4%,准确率达95.6%,阴性预测值为98.7%,阳性预测值为90.9%。结论:血清AMA-M2检测有较高的诊断效率,可作为PBC诊断的重要血清免疫学指标。     

The E1 alpha and beta subunits of the pyruvate dehydrogenase complex are M2'd' and M2'e' autoantigens in primary biliary cirrhosis

1. Sera from 76 patients with primary biliary cirrhosis (PBC) and 66 control subjects (53 with chronic liver disease and 13 healthy normal women) were immuno-blotted against purified E1 component of bovine pyruvate dehydrogenase complex (PDC) and bacterial PDC. 2. Thirty-one out of seventy-six (41%) sera from PBC patients showed a positive response to bovine E1 alpha, and five of these 31 (7% of total) reacted with bovine E1 beta. None of the control sera reacted with bovine E1 alpha or beta. 3. None of the PBC sera that recognized bovine E1 subunits reacted with bacterial PDC E1. 4. In the PBC patients there was no correlation between presence of antibodies to E1 alpha and beta subunits and histological stage of the disease. 5. Our data demonstrate that the E1 alpha and beta components of mammalian PDC are the M2'd' and 'e' mitochondrial autoantigens, respectively.     

临床14 282份标本自身抗体谱检测结果分析

目的 探讨就诊人群中抗核抗体（ANA）、抗心肌抗体（HMA）、抗胃壁细胞抗体（PCA）、抗平滑肌抗体（SMA）、抗肝肾微粒体抗体（LKMA）、抗线粒体抗体（AMA）、抗线粒体抗体M2亚型（AMA—M2）等自身抗体的阳性分布趋势，及其在不同疾病中的分布与疾病诊断的价值。方法 用间接免疫荧光法检测14282份临床血清标本中ANA、HMA、PCA、SMA、LKMA和AMA，用酶联免疫吸附测定（ELISA）检测AMA—M2亚型；结合患者临床资料分析这些自身抗体在不同年龄、性别和疾病中的阳性分布趋势及临床应用价值。结果 ANA、HMA、PCA、SMA、LKMA、AMA和AMA-M2在14282例就诊人群的阳性率分别为：41．23％、0．19％、3．26％、10．25％、0．11％、11．90％和6．96％。女性中ANA、AMA和AMA-M2阳性率显著高于男性（P〈0．01），人群中ANA、PCA、SMA、AMA和AMA—M2阳性率随年龄增加而升高（SMA为P〈0．05，其余P〈0．01）。在系统性硬皮病（SSc）和系统性红斑狼疮（SLE）ANA阳性率达94．6％和93．9％，自身免疫性肝炎（AIH）中SMA阳性率64．5％，原发性胆汁性肝硬化（PBC）中AMA和AMA-M2阳性率最高，分别为83．2％、70．2％，但其他疾病中也有5．4％-22．6％和1．8％-12．9％不等的阳性率。结论 SLE、SSc、类风湿关节炎（RA）、干燥综合征（SS）和肌炎／皮肌炎（PM／DM）患者中的主要阳性抗体为ANA，AIH患者主要为ANA和SMA阳性，PBC患者主要为ANA、AMA和AMA—M2阳性。但是，同一种自身抗体可以出现在不同的自身免疫性疾病（AID），如AMA可出现在AIH、SS和PM／DM中；一种AID可同时出现多种自身抗体，如AIH息者可出现ANA、SMA、AMA和AMA—M2同时阳性。因此，在临床诊疗中应综合分析，重视抗体滴度在AID诊疗中的作用，以避免片面根据某种自身抗体阳性所致的误诊。     

用重组M2三联体抗原建立原发性胆汁性肝硬化免疫检测法

目的 建立原发性胆汁性肝硬化（PBC）特异性免疫学检测方法。方法 在重组质粒表达的基础上，用亲和层析进一步纯化重组蛋白后，用酶免疫吸附法检测M2抗体。结果 在PBC组11例患者哈部检出M2抗体，阳性率为1005，而非PBC组75例患者中无一检出M2抗体，本法与病理检查和临床诊断的相关性有非常显著意义（P＜0.01）。结论 本法检测M2抗体有较好的敏感性及特异性，为PBC的早期发现和临床诊断提供了有力的工具。     

抗线粒体抗体阳性患者的AMA-M2亚型检测及ANA核型分析

目的了解不同滴度抗线粒体抗体(AMA)阳性患者AMA M2亚型抗体(AMA-M2)浓度分布状况及在原发性胆汁性肝硬化(PBC)患者中的阳性率及相伴ANA核型。方法采用间接免疫荧光法检测自身抗体,采用酶联免疫吸附试验检测502例AMA阳性标本中的AMA-M2,并对已明确诊断的71例PBC患者进行AMA-M2亚型及ANA核型分析。结果在502例AMA阳性患者中AMA-M2阳性率为76.1%;71例PBC中95.8%AMA抗体效价大于1:160、90.1%AMA抗体效价大于1:320、73.2%AMA抗体效价大于1:640。95.8%AMA-M2阳性:67.6%M2200 RU/ML、84.5%M2100 RU/ML,并有56.3%存在ANA。结论 PBC中AMA、AMA-M2呈高滴度、高浓度集中,高效价AMA、AMA-M2及ANA的部分核型有助于PBC的诊断。     

原发性胆汁性肝硬化85例实验室检测指标的分析

目的探讨原发性胆汁性肝硬化(PBC)的免疫学实验诊断特点。方法观察85例PBC患者的临床表现,并分析有关实验室检查资料。结果本组患者临床症状不典型。所有患者γ-谷氨酰转移酶(GGT)升高,98.8%(84/85)的患者碱性磷酸酶(ALP)升高,87.1%(74/85)的患者总胆红素(TB il)和结合胆红素(CB il)升高,81.2%(69/85)的患者丙氨酸转氨酶(ALT)、78.8%(67/85)的患者门冬氨酸转氨酶(AST)、52.9%(45/85)的患者球蛋白(GLB)、95.7%(22/23)的患者总胆汁酸(TBA)、84.0%(21/25)的患者高密度脂蛋白胆固醇(HDL-C)升高。自身抗体检测有59例抗核抗体(ANA)阳性(69%),其中32例为核膜型,20例为着丝点型;85例患者抗线粒体抗体(AMA)均为阳性,其中80例(94.1%)AMA M2阳性。结论PBC无典型性临床症状,医生可以通过临床表现结合AMA,尤其是AMA M2的检测结果获得诊断信息。     

M2抗体重组人源靶抗原二联体在原发性胆汁性肝硬化患者中的检测及应用

目的 利用重组抗原BCOADA-E2和PDC-E2的二联体（BP），检测PBC患者血清中的M2抗体并探讨其临床意义。方法 经Ni—NTA亲和柱纯化重组表达的BP融合蛋白，分别建立免疫印迹法（IBT）和酶链免疫吸附（ELISA）法检测60份PBC患者血清，以60份其他肝病患者、60份自身免疫病患者、80例正常人血清为对照组。结果 经常规试剂盒检测为M2（＋）的60份患者血清，利用重组抗原检测阳性53例，阴性7例，阳性率为88．3％；经常规试剂盒检测为M2（-）的60份自身免疫病患者血清、60份其他肝病患者和80例正常人血清，利用重组抗原检测为M2（-）。结论 利用重组抗原BP检测M2抗体敏感性较高。对临床辅助诊断PBC提供一定的手段。     

Enzyme-linked immunoadsorbent assay for the detection of cytomegalovirus-IgM: comparison between eight commercial kits, immunofluorescence, and immunoblotting.

Eight commercially available enzyme-linked immunoadsorbent assays (ELISA) for the detection of cytomegalovirus (CMV)-specific IgM were used in parallel to determine the presence of CMV-IgM in 123 serum samples from pregnant women. The results obtained with the eight kits were compared. Based on concordance of six or more of the eight kits, we assessed sensitivity, specificity, and overall agreement, as well as incidence of false-positive and -negative results for each kit. The results obtained by ELISA were then compared with those obtained by immunofluorescence (IF) and immunoblotting (IB). Our study did not single out one outstanding ELISA kit among the eight evaluated, nor did it suggest that IF or IB are better than ELISA. Furthermore our results indicate that IB might be useful in several cases as, beside its good sensitivity, most IB-false-positive sera are easily recognized as reacting exclusively with pp150, the unique reactivity to pp150 not being among the IB profiles of IB-true-positive sera. Nevertheless 14.6% of sera remained CMV-IgM-indeterminate.     

原发性胆汁性肝硬化所致肝癌患者自身抗体特征分析

目的 探讨原发性胆汁性肝硬化（PBC）合并原发性肝癌（HCC）患者的自身抗体特征分析.方法 回顾性分析2007-2012年于我院住院治疗PBC患者350例,对这些患者抗线粒体抗体（AMA）、抗线粒体抗体M2亚型（AMA-M2）、抗核抗体（ANA）、抗平滑肌抗体（SMA）、抗肝肾微粒体抗体（LKM）滴度及ANA的核型分析.结果 350例PBC患者发展为肝癌为2.57%（9/350）,其中女性患者2.38%（7/293）,男性患者3.5%（2/57）,9例患者中有7例可见AFP值升高,8例患者可见AMA、AMA-M2阳性,50%呈AMA高滴度（≧1：320）阳性.100%患者ANA检测结果阳性,88.9%患者滴度以高滴度为主（≧1：320）.ANA核型以混合核型为主.抗核抗体谱检测可见3例患者分别为CENPB、SSA、Scl-70阳性,2例患者gp210阳性.结论 PBC患者中有2.57%发展为HCC,自身抗体检测情况与PBC患者类似.多数患者可检测出高滴度的AMA、AMA-M2及ANA,这些患者ANA核型以混合核型为主,患者血清AFP值与自身抗体有一定相关性,可能提示血清AFP值与自身免疫反应强弱相关.     

AMA-M2阳性的原发性胆汁性肝硬化患者免疫学特点分析

目的 分析抗线粒体M2亚型抗体阳性（AMA-M2）的原发性胆汁性肝硬化（PBC）患者免疫学等实验室诊断特点,推进临床PBC的早期诊断和治疗.方法 对400例AMA-M2阳性的PBC患者采用间接免疫荧光法检测血清ANA、AMA、SMA、LKM和PCA等5种自身抗体.采用免疫印迹法检测患者血清7种主要肝抗原：Sp100、PML、gp210、LKM-1、LC-1、SLA/LP和Ro-52.应用全自动生化分析仪检测患者ALP、γ-GT等生化指标,全自动血细胞分析仪和凝血分析仪检测患者WBC、RBC、HGB、PLT和PT等血液学指标.结果患者自身抗体检测：ANA荧光模型主要包括核膜型、多核点型、着丝点型和胞浆颗粒型,阳性率分别为70％、52％、43％、3％.荧光模型组合方式包括：多核点型＋核膜型、着丝点型＋核膜型、着丝点型、多核点型、胞浆颗粒型以及仅AMA阳性者,阳性率分别为42％、28％、15％、10％、3％、2％,荧光滴度均≥1：320.SMA、PCA、LKM阳性率均低于5％.患者主要肝抗原为Ro-52、gp210、PML和Sp100,阳性率分别为53％、44％、43％、24％,荧光滴度≥1∶320,而LKM-1、LC-1、SIA/LP阳性率均低于5％.PML、Sp100及Sp100＋ PML与gp210的阳性一致率分别为29％、11％、7％.临床血液检测结果显示有41％的患者WBC和RBC降低,53％的患者HGB降低,38％的患者PLT降低,而有42％的患者PT延长.生化检测结果显示有96％和93％的患者ALP和γ-GT升高.结论 AMA-M2阳性的PBC患者具有高滴度、特征性自身抗体荧光模型及自身肝抗原,结合临床血液及生化检测结果将为临床诊断提供重要帮助.     

A contemporary perspective on the molecular characteristics of mitochondrial autoantigens and diagnosis in primary biliary cholangitis

Primary biliary cholangitis (PBC) is an autoimmune hepatobiliary disease characterized by immune mediated destruction of the intrahepatic small bile ducts and the presence of antimitochondrial antibodies (AMAs). The mitochondrial autoantigens have been identified as the E2 subunits of the 2-oxo-acid dehydrogenase complex, including the E2 subunits of pyruvate dehydrogenase, branched-chain 2-oxo acid dehydrogenase complex, oxoglutarate dehydrogenase complex, E3 binding protein and PDC E1 alpha subunit. The AMA epitope is mapped within the E2 lipoic acid binding domain, which is particularly important for oxidative phosphorylation. In addition, lipoic acid, which serves as a swinging arm to capture electrons, is particularly susceptible to an electrophilic attack and may provide clues to the etiology of PBC. This review emphasizes the molecular characteristics of AMAs, including detection, immunochemistry and the putative role in disease. These data have significance not only specifically for PBC, but generically for autoimmunity.     

原发性胆汁性胆管炎患者血清自身抗体及生化指标的特征分析

目的 探讨原发性胆汁性胆管炎(PBC)患者血清自身抗体及生化指标的特征及临床意义。方法 采用间接免疫荧光法检测64例PBC,81例病毒性肝病,50例健康体检者三组血清中的抗核抗体(ANA)、抗线粒体抗体(AMA); 采用免疫印迹法检测三组的自身免疫肝病抗体(AMA-M2,M2-3E,抗Sp100抗体,抗PML抗体,抗gp210抗体,抗LKM-1抗体,抗LC-1抗体,抗SLA/LP 抗体,Ro52); 采用全自动生化分析仪检测其生化指标。结果 PBC组ANA和AMA阳性率明显高于病毒性肝病组,差异有统计学意义(χ2 =56.07,54.72,均P<0.01); PBC组自身免疫肝病抗体(AMA-M2,M2-3E,抗Sp100抗体,抗gp210抗体,Ro52)阳性率与病毒性肝病组比较,差异有统计学意义(χ2=9.06～98.70,均P<0.01); PBC组生化指标丙氨酸氨基转移酶(ALT)与病毒性肝病组比较,差异无统计学意义(F=6.069,P>0.05),其余总胆红素(TBIL)、天冬氨酸氨基转移酶(AST)、碱性磷酸酶(ALP)和谷氨酰转肽酶(GGT)、总胆固醇(TC)与病毒性肝病组及健康对照组比较差异均有统计学意义(F=6.069～35.56,均P<0.05); AMA-M2阳性和阴性的PBC患者之间的生化指标比较差异无统计学意义(F=0.425～2.775,P>0.05); 抗gp210抗体阳性患者的TC高于阴性患者,差异有统计学意义(F=4.521,P=0.044)。结论 自身抗体ANA,AMA,AMA-M2,M2-3E,抗Sp100抗体,抗gp210抗体有助于PBC的早期诊断,结合生化指标则有利于PBC和病毒性肝病的鉴别诊断和疗效观察。此外,抗gp210抗体阳性与PBC患者的TC有一定的相关性。     

Anti-mitochondrial antibody immunofluorescent titres correlate with the number and intensity of immunoblot-detected mitochondrial bands in patients with primary biliary cirrhosis

BACKGROUND: Primary biliary cirrhosis (PBC)-specific anti-mitochondrial antibodies (AMA), routinely detected by indirect immunofluoresnce, immunofix various mitochondrial antigens by immunoblot (IB). We have investigated whether there is a relationship between IFL-determined AMA titres and the number, intensity or pattern of IB-bands. METHODS: By computer-assisted imaging technology, an IB analysis using human liver mitochondrial preparation was performed in 100 AMA positive PBC samples, subdivided in 5 groups each consisting of 20 cases with very high, high, intermediate, low or borderline IFL titres and in 19 AMA-negative cases. RESULTS: Reactivity to at least 1 mitochondrial band (range 1-12) was present in 114 (96%) cases. Reactivity to the major pyruvate dehydrogenase E2-subunit 74-kDa autoantigen positively correlated with AMA IFL titres but was absent in 25% IFL-positive cases. The intensity and number of IB-bands differed significantly among groups (p<0.001). IFL AMA titres positively correlated with the total number (r=0.8, p<0.001) and the intensity of the IB-bands (r=0.75, p<0.001). CONCLUSIONS: Our study indicates that IFL-determined AMA relate to the number and intensity of immunofixed bands. Reactivity to PDC-E2 does not correlate with IFL titres in a considerable proportion of patients suggesting that detection methods of AMA need to be based in the entire spectrum of the disease-specific mitochondrial antigens.     

自身免疫性肝病148例临床特点分析

目的 分析自身免疫性肝病（autoimmune liver diseases,ALDS）的临床、生化及自身抗体特点.方法 分析我院1996年-2005年148例ALDS患者其中自身免疫性肝炎（autoimmune hepatitis,AIH）患者52例,原发性胆汁性肝硬化（primary biliary cirrhosi,PBC）患者96例的临床表现,并分析有关实验室检查资料.结果 两组患者临床症状均不典型,肝功能各项指标普遍异常,AIH组以ALT升高更为明显,而PBC组患者ALP和GGT升高更为显著（P＜0.01）.在自身抗体检测情况两组有极大的差异.AIH患者主要以高效价的抗核抗体（antinuclear antibodies,ANA）（44/52）和/或抗平滑肌抗体（antismooth muscle antibodies）（35/52）为主,此外,尚有9.6%（5/52）和3.8%（2/52）的AHI患者出现抗可溶性肝抗原抗体（auti-soluble liver antigen autoantibdies,抗SLA）和抗肝/肾微粒体（liver/kidney microsomal,LKM）抗体阳性.PBC组以抗线粒体抗体（mitahondrial anto-antibodies,AMA）阳性（99.0%）为主,93.8%的患者AMAM2阳性.结论 AIH及PBC的诊断可结合临床、生化、自身抗体检查的结果,其中自身抗体的检测尤为重要.