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     

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.     

Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid

Patients with primary biliary cirrhosis (PBC) may have additional features of autoimmune hepatitis (AIH). Corticosteroids usually contraindicated in PBC have been advocated for these patients. Patients with antimitochondrial antibody (AMA)-positive PBC from two previous randomized, controlled trials were assessed for features of AIH. Their biochemical, immunologic, and histologic responses to ursodeoxycholic acid (UDCA) versus placebo were compared with those without AIH features. The survival of patients testing positive or negative for antinuclear antibodies (ANA) was also examined. Features of AIH were defined by the presence of 2 or more of the following: 1) alanine transaminase (ALT) > 5 x the upper limit of normal (ULN); 2) immunoglobulin G (IgG) > 2 x ULN or positive anti-smooth muscle antibody (ASMA); and 3) moderate to severe lobular inflammation on pretreatment liver biopsy. Testing for AMA, ASMA, and ANA was done by immunofluorescence. The change in serum bilirubin, alkaline phosphatase (ALP), transaminases, IgM, and IgG from baseline to 2 years was compared. Of the 331 patients randomized, 16 (4.8%) had features of AIH (12 UDCA, 4 placebo). The median percent change in serum biochemistry and immunoglobulin values were similar in patients with PBC +/- features of AIH after 2 years of therapy with UDCA. Over 2 years, little change in histologic features of AIH was observed. Survival was similar for patients with PBC with and without ANA. In conclusion, features of AIH in PBC may be transient and response to UDCA therapy similar to patients with PBC without features of AIH.     

Polymyositis associated with autoimmune hepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura

We describe a 40-year-old woman with polymyositis (PM) who developed autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and autoimmune thrombocytopenic purpura (AITP) concurrently. About 4 years earlier, she suffered from muscle weakness probably due to PM. When she visited our hospital, she had polyarthritis, myalgia, symmetrical proximal limb-muscle weakness, elevated muscle enzymes, and myogenic abnormalities on electromyogram. Pathological findings obtained by muscle biopsy showed histological findings consistent with PM. Her serum liver enzymes were also elevated. The histology obtained by liver biopsy revealed the mixture findings of chronic active hepatitis and biliary cirrhosis. As antibodies to mitochondria M2 and liver/kidney microsome type 1 (LKM-1) were present, we concluded her liver disease was due to an overlap of AIH and PBC. Furthermore, purpura on the legs with thrombocytopenia appeared in parallel with liver dysfunction. She was diagnosed as having AITP by clinical and laboratory findings. Her serum showed a speckled pattern in immunofluorescence antinuclear antibody testing, but the antigen specificities were distinct from those of the known myositis-related autoantigens. This is a first case report of PM accompanied by AIH, PBC, and AITP. It was notable that there was an overlap of disease-associated immunological findings and immunogenetic backgrounds. This case provides a possible insight into the mechanisms and interplay of autoimmune diseases.     

Polymyositis associated with autoimmune hepatitis,primary biliary cirrhosis,and autoimmune thrombocytopenic purpura

Abstract

We describe a 40-year-old woman with polymyositis (PM) who developed autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and autoimmune thrombocytopenic purpura (AITP) concurrently. About 4 years earlier, she suffered from muscle weakness probably due to PM. When she visited our hospital, she had polyarthritis, myalgia, symmetrical proximal limb-muscle weakness, elevated muscle enzymes, and myogenic abnormalities on electromyogram. Pathological findings obtained by muscle biopsy showed histological findings consistent with PM. Her serum liver enzymes were also elevated. The histology obtained by liver biopsy revealed the mixture findings of chronic active hepatitis and biliary cirrhosis. As antibodies to mitochondria M2 and liver/kidney microsome type 1 (LKM-1) were present, we concluded her liver disease was due to an overlap of AIH and PBC. Furthermore, purpura on the legs with thrombocytopenia appeared in parallel with liver dysfunction. She was diagnosed as having AITP by clinical and laboratory findings. Her serum showed a speckled pattern in immunofluorescence antinuclear antibody testing, but the antigen specificities were distinct from those of the known myositis-related autoantigens. This is a first case report of PM accompanied by AIH, PBC, and AITP. It was notable that there was an overlap of disease-associated immunological findings and immunogenetic backgrounds. This case provides a possible insight into the mechanisms and interplay of autoimmune diseases.     

慢性肝病中抗肝抗原自身抗体的检测

目的　探讨中国人不同病因所致慢性肝病患者中抗肝抗原自身抗体的存在状况及自身免疫性肝病的自身抗体特征。方法　 166例肝功能异常患者分为 6组 :自身免疫性肝炎 (AIH ) 12例、原发性胆汁性肝硬化 (PBC) 2 0例、原发性硬化性胆管炎 (PSC)13例、HBV组 66例、HCV组 2 2例、肝豆状核变性 (HDL) 3 9例。用间接免疫荧光法检测抗核抗体 (ANA)、平滑肌抗体 (SMA)、抗肝肾微粒抗体I型抗体 (anti LKM1)、抗线粒体抗体 (AMA)和抗中性粒细胞胞浆抗体 (ANCA) ,免疫印迹法检测抗肝细胞胞溶质抗原 1型抗体 (anti_LC1)、抗可溶性肝抗原 /肝胰抗原抗体 (anti_SLA/LP)、抗肝肾微粒抗体 1型 (anti_LKM1)、AMA_M2亚型等多种肝抗原自身抗体。结果　 166例中ANA、AMA、M_2、pANCA阳性率在 7组中有显著差异 (P <0 .0 1)。PBC中AMA、M 2阳性检出率均为 10 0 % ,PSC中pANCA阳性检出率为 5 3 8% ,Fisher精确检验在a =0 .0 0 2水准与其他各组比较有显著差异。AIH与PBC的ANA阳性率分别为10 0 %和 60 % ,Fisher精确检验在a =0 .0 0 2水准二者无显著差异。与其他各组比较有显著差异。在AIH组SMA阳性率为 2 5 % ,LKM 1、LC 1、SLA/LP阳性率均为 8.3 % ,统计学处理与其他组无显著差异 (P >0 0 5 ) ,可能与病例少有关。PBC中分别有 1     

不明原因肝功能异常患者中自身免疫性肝病的临床分析

背景:自身免疫性肝病(AILD)起病隐匿,临床表现无特异性,早期诊断十分困难。目的:总结不明原因肝功能异常患者随访过程中AILD的诊断情况。方法:回顾性分析161例不明原因肝功能异常患者的临床随访结果,包括AILD相关自身抗体监测,部分患者经本人同意后行肝穿刺活检病理检查。结果:在6个月～6年的随访中,57例患者诊断为AILD,其中自身免疫性肝炎(AIH)28例,原发性胆汁性肝硬化(PBC)21例,PBC-AIH重叠综合征8例。初次入院时,AILD患者临床表现与非AILD患者无明显差异,但无症状者所占比例较大;血生化指标中的GGT、ALP、球蛋白水平、免疫相关指标中的IgG、IgM水平均显著高于非AILD患者。AILD患者自身抗体ANA、AMA/AMA-M2、SMA阳性率显著高于非AILD患者,肝活检病理检查结果与相应疾病的组织病理学改变相符。结论:对于不明原因的反复肝功能异常患者,定期监测AILD相关自身抗体和肝活检病理有助于早期诊断、早期治疗AILD。     

血清抗线粒体抗体阳性患者20例临床及实验室指标分析

目的分析血清抗线粒体抗体（AMA）阳性患者的临床及实验室指标,探讨抗线粒体抗体的诊断价值。方法将AMA阳性患者20例,分为原发性胆汁性肝硬化（PBC）11例和非PBC9例,分析每组临床特点、抗核抗体、肝功能指标。结果20例AMA阻性患者具有相同的抗核抗体核型,即胞浆粗颗粒型,滴度为1：320～1：1000;PBC患者,ALP、γ-GT较ALT、AST显著增高,而并发其他自身免疫性疾病的PBC患者,临床特征不典型,临床不易诊断;9例非PBC患者中,肝病患者6例,其他无肝病患者3例。结论AMA在PBC患者中检出率极高。但AMA并不只出现在PBC患者血清中,在肝胆疾病、其他疾病患者血清中亦可检出。     

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.     

Consecutive occurrence of primary biliary cirrhosis and autoimmune hepatitis: a case report and review of the literature

Autoimmune cholangiopathy is a term that describes a subset of patients with overlapping features of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AH). These patients typically have cholestasis, negative antimitochondrial antibody (AMA), liver histology suggestive of PBC, and may respond clinically to corticosteroid therapy. We describe a patient who presented with typical AMA-positive, biopsy-proven PBC who responded to ursodeoxycholic acid therapy both clinically and biochemically. Approximately 3 yr later, she developed elevated transaminases with biopsy-proven antinuclear antibody (ANA) negative AH. The AMA was negative at this time. After responding to steroid therapy for the AH and after discontinuing the ursodeoxycholic acid, the patient had a clinical recurrence of PBC with renewed AMA positivity.     

Primary biliary cirrhosis and type II autoimmune polyglandular syndrome.

A 45-year-old female was diagnosed with Hashimoto's thyroiditis in 1976 and Addison's disease in 1979. At that time, her antimitochondrial antibody (AMA) level was elevated at 1:32. She subsequently developed premature ovarian failure and type I diabetes mellitus. In 1996, she became jaundiced with a cholestatic enzyme pattern. AMA was positive at a titre of 1:256. A liver biopsy confirmed the diagnosis of primary biliary cirrhosis (PBC). She underwent a liver transplantation in January 1998. This is the first report of PBC in association with type II autoimmune polyglandular syndrome. The association of PBC with other organ-specific autoimmune diseases supports an immune-mediated pathogenesis and may have implications in further studies of PBC.     

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.     

Anti-mitochondrial antibody positive autoimmune hepatitis triggered by EBV infection in a young girl

Autoimmune hepatitis (AIH) is rare in children. Two types of childhood autoimmune hepatitis are recognized: AIH type 1 which is characterized by the presence of smooth muscle (SMA) and/or antinuclear (ANA) antibodies; and AIH type 2 which is positive for anti-liver kidney microsomal type 1 (anti-LKM-1) antibody. Anti-mitochondrial antibody (AMA) is considered the hallmark for primary biliary cirrhosis (PBC) that occurs primarily in adult women and is characterized by destruction of the intralobular bile ducts and progression to cirrhosis and liver failure. Antimitochondrial-antibody-positive AIH is extremely rare in children. We report a 13 year old Saudi girl with type-1 AIH who had a strongly positive anti-mitochondrial antibody and no evidence of small bile duct disease in the liver biopsy.     

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。     

Autoimmune cholangitis and primary biliary cirrhosis--an autoimmune enigma

AIMS/BACKGROUND: Autoimmune cholangitis/cholangiopathy (AIC) is an enigmatic disease marked by chronic cholangitis, antinuclear antibodies (ANA) and sero-negativity for conventionally detected antimitochondrial antibodies (AMA). We examined whether AIC is a distinct entity, an AMA-negative variant of primary biliary cirrhosis (PBC), or a cholangiopathic variant of autoimmune hepatitis (AIH) by comparing the clinical, laboratory and autoantibody profiles of 21 cases of AIC, 37 cases PBC and 16 cases of AIH from selected Japanese patients. METHODS: The specificities of AMA and ANA were determined by immunofluorescence, immunoblotting and enzyme inhibition assays using various mitochondrial and nuclear autoantigens, and the frequencies for these groups were compared. RESULTS: By clinical, biochemical and histological data, AIC and PBC were similar and both were clearly distinct from AIH. Serologically, by immunofluorescence of AMA and ANA, there was polarisation. By immunoblotting, and notwithstanding the negative test for AMA, a proportion of the AIC sera reacted with the E2 subunits of the 2-oxo-acid dehydrogenase enzyme complexes, but more particularly with the lower molecular weight E2 subunits. The antinuclear reactivity in AIC was with centromere, Sp100 and nuclear pore complex proteins as in PBC, but preferentially with the nuclear pore complex. CONCLUSION: Our results demonstrate that AIC and PBC are similar diseases. However this duo is of interest because, usually, among sets of autoimmune syndromes, differences in serological targetting are matched by differences in clinical presentation: AIC and PBC are an exception to this.     

Diagnosing autoimmune hepatitis in nonalcoholic fatty liver disease: is the International Autoimmune Hepatitis Group scoring system useful?

Background There are no surrogate serum markers for autoimmune hepatitis (AIH) and nonalcoholic fatty liver disease (NAFLD). An AIH scoring system was reported by the International Autoimmune Hepatitis Group; however, the criteria did not focus on making the distinction between AIH and NAFLD. We examined the effectiveness of using the AIH score for diagnosing AIH in NAFLD patients. We also identified the prevalence of autoimmune phenomena, in terms of various auto-antibodies, including antinuclear antibodies (ANA), to determine whether these markers had any clinicopathological significance, and whether they were related to the patients' clinical courses. Methods We studied 212 patients (103 males and 109 females) with biopsy-proven NAFLD. The AIH score of each patient was calculated without including the liver biopsy results. The patients were divided into three groups based on their clinicopathological features: the overlap group (those with clinical and histological features of both NAFLD and AIH), the systemic group (those with systemic antoimmune disease other than AIH), and the “other” group (patients with no antoimmune disease). To evaluate the clinicopathological significance of ANA in NAFLD patients, those without autoimmune diseases (the “others” group) were classified according to their ANA positivity and ANA titer. Results Seventy patients (33.0%) were positive for ANA. Among the female patients, 106 patients (97.2%) had an AIH score of 10 or more. Of the 103 male patients, 21 (20.4%) had an AIH score of 10 or more. However, after liver biopsy, only 1 patient (0.5%) could be classified as “definite AIH.” In the NAFLD patients without autoimmune diseases (“other” group), multivariate logistic regression analysis found that female sex was an independent predictor of the presence of ANA (P = 0.029). In contrast, multivariate logistic regression analysis found that severe obesity (body mass index [BMI], ≥30 kg/m²) was the only independent predictor of the presence of an ANA titer of 1 : 80 or more (P = 0.026). Conclusions The AIH score without liver biopsy findings was not useful for diagnosing AIH in NAFLD patients. In patients with elevated ANA titers and risk factors for NAFLD, it is very important to perform a liver biopsy to make a definitive diagnosis before treatment.     

187例自身免疫性肝病的临床特点分析

目的分析自身免疫性肝病（AILD）的临床、生化及免疫学特点。方法分析北京大学人民医院2001年1月～2010年12月187例自身免疫性肝病患者的临床表现、生化及免疫学特点。结果 AILD的发病以40岁以上女性多见,30.5%（57/187）的患者确诊时已进展为肝硬化失代偿期。临床症状无特异性,自身免疫性肝炎（AIH）以ALT、IgG及γ球蛋白升高更为明显（P〈0.05）,而PBC以ALP、GGT、IgM升高更为明显（P〈0.05）。AILD患者常合并一种或多种肝外自身免疫病,AILD合并干燥综合症最为常见,AIH组及PBC组合并干燥综合症的发生率均达30%以上。AILD患者均有一种或多种自身抗体阳性,其中AIH组ANA阳性率为88.3%,AMA阳性率为7.5%（3/40）,AMA-M2阳性3.7%（2/54）;PBC组ANA阳性率为88.2%（97/110）,AMA阳性率为96.4%（81/84）,AMA-M2阳性率为94.4%（85/90）。AIH患者主要应用糖皮质激素治疗,其中共46例（59.7%）患者单用或联用UDCA治疗后肝脏酶学指标改善。结论生化、免疫学、自身抗体等检查对诊断与鉴别诊断具有重要意义。UDCA在AIH的治疗中有一定的作用。     

Autoimmune cholangitis and primary biliary cirrhosis — an autoimmune enigma

Abstract: Aims/Background: Autoimmune cholangitis/cholangiopathy (AIC) is an enigmatic disease marked by chronic cholangitis, antinuclear antibodies (ANA) and sero-negativity for conventionally detected anti-mitochondrial antibodies (AMA). We examined whether AIC is a distinct entity, an AMA-negative variant of primary biliary cirrhosis (PBC), or a cholangiopathic variant of autoimmune hepatitis (AIH) by comparing the clinical, laboratory and autoantibody profiles of 21 cases of AIC, 37 cases of PBC and 16 cases of AIH from selected Japanese patients. Methods: The specificities of AMA and ANA were determined by immunofluorescence, immunoblotting and enzyme inhibition assays using various mitochondrial and nuclear autoantigens, and the frequencies for these groups were compared. Results: By clinical, biochemical and histological data, AIC and PBC were similar and both were clearly distinct from AIH. Serologically, by immunofluorescence of AMA and ANA, there was polarisation. By immunoblotting, and notwithstanding the negative test for AMA, a proportion of the AIC sera reacted with the E2 subunits of the 2-oxo-acid dehydrogenase enzyme complexes, but more particularly with the lower molecular weight E2 subunits. The antinuclear reactivity in AIC was with centromere, Sp100 and nuclear pore complex proteins as in PBC, but preferentially with the nuclear pore complex. Conclusion: Our results demonstrate that AIC and PBC are similar diseases. However this duo is of interest because, usually, among sets of autoimmune syndromes, differences in serological targetting are matched by differences in clinical presentation: AIC and PBC are an exception to this.     

25例原发性胆汁性肝硬化患者临床和病理特征分析

目的通过分析原发性胆汁性肝硬化患者的临床资料,旨在提高对该病的诊治水平。方法对25例原发性胆汁性肝硬化患者的临床和病理资料进行回顾性分析,总结患者的临床特征、各项生化指标、免疫学与病理学特点。结果在25例PBC患者,ALP均明显升高(524.9±214.4U/L),17例γ-GT升高(431.1±248.9U/L),8例总胆红素升高(274.1±161.6μmol/L),均以直接胆红素升高为主,21例抗核抗体(ANA)阳性;12例行肝穿刺病理检查,其中11例符合PBC;8例影像学检查显示肝内胆管扩张,走行僵直。结论PBC主要累及中老年女性,血清ALP、γ-GT水平升高,AMA阳性以及病理学与影像学检查有助于诊断本病。     

Chronic active hepatitis with histological features of primary biliary cirrhosis

Summary A 51-year-old woman was evaluated because of Raynaud's phenomenon, Sjögren's syndrome, and general malaise. There was neither skin itching nor jaundice. Endoscopic retrograde cholangiopancreatography showed a normal extrahepatic as well as intrahepatic biliary tree. Serum GOT and GPT fluctuated with episodes of marked increases. The alkaline phosphatase was slightly increased and total cholesterol was normal. There were marked increases of IgG and IgM. AMA was positive at a titer of 1:320, which was measured by an indirect immunofluorescence method. PBC-specific AMA (anti-M₂) was positive, but mixed-form AMA (anti-M₄) negative. An LE-cell test, ASMA, ANA, and anti-DNA antibody were all positive on several repeated tests. A wedge liver biopsy tissue showed mixed features of PBC and CAH. A diagnosis of CAH was made on the basis of the clinical, serological, and morphologic findings. The patient responded well to prednisolone treatments with prominent improvement of her symptoms and liver function tests. Subsequently, AMA fell to undetectable levels by indirect immunofluorescence method.