自身免疫性肝炎

自身免疫性肝炎赵超叶光福曲军平何维熊广东省中山市博爱医院消化内科５２８４０３Ｓｕｂｊｅｃｔｈｅａｄｉｎｇｓｈｅｐａｔｉｔｉｓ／ｉｍｍｕｎｏｌｏｇｙ；ａｕｔｏｉｍｍｕｎｅｄｉｓｅａｓｅｓ；ａｕｔｏａｎｔｉｂｏｄｉｅｓ／ａｎａｌｙｓｉｓ主题词肝炎／免...     

三种自身免疫性肝病的临床特点比较

目的分析比较三种自身免疫性肝病(ALD)的临床表现、血清学和病理学特点及诊断意义。方法回顾性总结北京协和医院1998—2002年78例自身免疫性肝病患者的临床资料。结果1．临床表现：ALD以中年女性多见，在自身免疫性肝炎(Am)和原发性硬化性胆管炎(PSC)患者中发热明显多于原发性胆汁性肝硬化(PBC)，皮肤瘙痒在PSC最为突出；2．实验室检查：PBC和PSC以ALP、GGT显著升高为主，AIH以ALT、AST升高为主，血清抗体检测在AIH中抗核抗体(ANA)阳性占80％，抗线粒体抗体M：亚型(AMA-M：)对PBC最具特异性；3．影像学检查：PSC患者80％有肝内外胆管的僵硬、狭窄、变细及局部扩张；4．病理学：共有41例行肝穿刺病理活检，病理结果提出明确诊断者17例(41．5％)。结论ALD临床表现复杂多样但也各有特点，血清抗体检测有助于AIH与PBC的诊断与鉴别诊断。胰胆管影像检查有助于PSC的确诊。病理学检查的诊断价值有一定的局限性。     

自身免疫性肝炎概述

本概述自身免疫性肝炎的病理学特征、发病机理、诊断及治疗。     

自身免疫性肝炎的研究进展

自身免疫性肝炎(autoimmune hepatitis,AIH)是一种呈慢性炎症性坏死的肝脏疾病,其发病与HLA等位基因密切相关.近20年来,AIH的诊断与治疗取得了较多进展,发现了更多的AIH自身抗体、基因及新型治疗药物,建立了AIH的血清免疫学检查方法和遗传异质性研究;掌握了AIH临床表现、发生发展、疾病转归与种族、地域分布和遗传背景的关系.本文就AIH有关研究进展作一综述.     

自身免疫性肝炎研究进展

自身免疫性肝炎研究进展青岛医学院教学医院青岛市人民医院内科池肇春自身免疫性肝炎（ＡＩＩＩ）是与自身免疫反应密切相关的一种慢性肝病。肝组织学改变与慢性活动性肝炎相一致，临床上有自身免疫反应的各种表现，如黄疸、发热、皮疹、关节炎等各种症状，高γ球蛋白血症...     

35例自身免疫性肝病的临床特点与分析

目的分析比较三种自身免疫性肝病(ALD)的临床表现、血清学和病理学特点及诊断意义.方法回顾性总结昆明医学院第二附属医院2003～2005年35例自身免疫性肝病患者的临床资料.结果①临床表现:ALD以中年女性多见,自身免疫性肝炎(AIH)和原发性硬化性胆管炎(PSC)在年龄上有显著差异,PSC的发热症状较AIH和原发性胆汁性肝硬化(PBC)明显,常伴有胆系感染;②实验室检查:AIH表现为球蛋白显著升高,同时AST、ALT及ALP、GGT升高显著,PSC的TB和DB水平最高,IgG在AIH、PSC中的浓度与PBC比较有显著性差异,明显增高.同时IgA在PSC中浓度与AIH、PBC比较有显著性差异,其浓度明显高于其它两种ALD,AIH患者ANA阳性检出率为75%,可能存在AIH-PSC重叠综合症,其发生率约占17.6%;③影像学检查:PSC患者100%有肝内、外胆管的僵硬、狭窄、变细或局部扩张;④病理学:共有6例行肝穿刺病理活检,病理结果提出明确诊断者2例(33.3%).结论ALD临床表现复杂多样但各有特点,血清免疫学及抗体检测有助于AIH与PBC的诊断和鉴别诊断,ERCP或MRCP有助于PSC的确诊,病理学检查的诊断价值有一定的局限性.     

鼠溃疡性结肠炎模型的观察

目的评价胎鼠结肠植入同系成年鼠所致溃疡性结肠炎（ＵＣ）模型．方法将胎鼠结肠植入同系鼠肾包膜下，观察胎鼠结肠和成年鼠结肠的组织学变化，并且采用微量细胞毒方法检查心脏血淋巴细胞或肠粘膜淋巴细胞对自身结肠上皮细胞的细胞毒指数．结果胎鼠结肠成功地植入２７只同系成年鼠的肾包膜下，７ｄ后植入的胎鼠结肠出现免疫排斥反应，表现为粘膜炎症和溃疡形成．还观察到宿主鼠结肠的自身免疫反应，表现为淋巴滤泡增多和炎性细胞浸润．２７只实验鼠的心脏血淋巴细胞或结肠粘膜淋巴细胞对自身结肠上皮细胞的细胞毒指数分别为４１６％±３８％，３８４％±２１％，而１５只正常对照鼠的相应指数分别为８２％±１９％，７０％±１７％，两者间的差异有显著性意义（Ｐ＜００１）．结论胎鼠结肠植入同系成年鼠能导致类似于ＵＣ的变化，其中淋巴细胞对自身结肠的细胞毒作用在细胞免疫反应中可能起重要作用     

自身免疫性肝炎概述

本文概述自身免疫性肝炎的病理学特征、发病机理、诊断及治疗。     

自身免疫性肝炎中的自身抗体及自身抗原

自身免疫性肝炎 (ＡＩＨ)是一组不明原因、具有相似临床表现的慢性免疫性肝病 ,以免疫学指标如高丙种球蛋白和自身抗体为特征。同时 ,它亦是一些激发因子、自身抗原、遗传倾向和免疫调节网络之间错综复杂的相互作用而打破自身耐受 ,由自身免疫反应导致的持续性肝细胞损害的结果。自 195 0年Ｗａｌｄｅｎｓｔｒｏｍ首次报道ＡＩＨ以来 ,根据血清自身抗体谱把ＡＩＨ分成 3个血清学亚型 :Ⅰ型ＡＩＨ ,抗核抗体 (ＡＮＡ )或 (和 )抗平滑肌抗体 (ＳＭＡ)阳性 ;Ⅱ型ＡＩＨ ,抗肝肾微粒体 (ＬＫＭ )Ⅰ型抗体阳性 ;Ⅲ型ＡＩＨ ,抗可溶性肝抗原 /肝胰…     

急性肝坏死大鼠NO水平的动态变化和作用

目的探讨一氧化氮（ＮＯ）在急性肝坏死大鼠模型中的浓度动态变化及其可能的作用机制．方法通过用化学药物促进或抑制急性肝坏死大鼠ＮＯ的合成，检测其外周血ＮＯ及ＡＬＴ，ＡＳＴ的浓度变化，并观察肝脏的病理变化．结果①急性肝坏死大鼠血清ＮＯ浓度６，１２，２４，４８ｈ显著高于正常对照（μｍｏｌ／Ｌ，９０±１３，１３７±３１，６６±１６，４４±１０ｖｓ１６±６，分别为Ｐ＜００１，Ｐ＜００１，Ｐ＜００１，Ｐ＜００５）；ＡＬＴ，ＡＳＴ也显著升高（ＡＬＴ，Ｕ／Ｌ，６９８±１０８，２０５６±６６８，８５８±２０８，１９６±３４ｖｓ３５±１３，Ｐ＜００１；ＡＳＴ，Ｕ／Ｌ，８０１±９４，３５７５±７１４，１２７２±２４２，１７５±１２７ｖｓ６５±２２，Ｐ＜００１）；②用ＮＡＭＥ抑制ＮＯ合成，降低了急性肝坏死大鼠外周血ＮＯ浓度，却显著升高了ＡＬＴ，ＡＳＴ浓度．③急性肝坏死大鼠应用ＮＯ合成底物后，与阳性对照组相比，未见ＮＯ显著升高，ＡＬＴ，ＡＳＴ也无显著变化．结论ＮＯ对ＤＧａｌｎ／ＬＰＳ诱导的急性肝坏死大鼠肝组织具有保护作用．     

自身免疫性肝炎免疫学治疗进展

近年来自身免疫性肝炎(AIH)以其独特的临床和病理学特征,受到越来越多的关注。但目前其发病率和临床特征仍不十分清楚,尚需对其进行系统深入的研究。探讨了AIH的流行病学、病因和发病机制、临床特征及其诊断和治疗进展。指出TNFα中和性抗体、利妥昔单抗、全反式维甲酸和调节性T淋巴细胞移植在临床试验或AIH动物模型中均被证明对AIH有疗效。     

自身免疫性肝炎的病理特点

本文简要介绍了自身免疫性肝炎（AIH）发病机制的进展,阐述了AIH活动期和缓解期的病理形态特点及缓解的病理诊断标准。AIH的病理组织学特点为淋巴浆细胞性界面炎。急性加重或急性发病可呈现明显的小叶中心带坏死,这些病变中浆细胞成簇是诊断AIH的重要依据。肝穿病理形态有助于澄清其他自身抗体阳性的慢性肝病。并介绍了IgG4相关性自身免疫性肝炎和原位自身免疫性肝炎的病理特点     

中药三甲益肝冲剂预防鼠肝纤维化

目的探讨中药三甲益肝冲剂对大鼠肝纤维化的预防作用．方法以四氯化碳造成大鼠肝纤维化模型，造模同时以三甲益肝冲剂（冲剂组）灌胃，观察其对大鼠肝纤维化的预防作用，并与促肝细胞生长素（ｐＨＧＦ）组和大黄庶虫虫丸预防组（虫丸组）比较．血清肝纤维化指标———透明质酸（ＨＡ）、Ⅳ型胶原（ⅣＣ）和层粘连蛋白（ＬＮ）用ＲＩＡ法测定，并作肝组织病理学检查．统计学处理分别采用方差分析和秩和检验．结果与模型组相比，冲剂组大鼠血清ＡＬＴ，ＡＳＴ，Ｇｌｂ，ＨＡ，ⅣＣ及ＬＮ均明显降低（Ｐ＜００１），肝纤维化程度降低（Ｐ＜００１）．与虫丸组相比，冲剂组大鼠血清ＨＡ及ＬＮ明显较低（Ｐ＜００５），倍量冲剂组ＡＬＴ降低（Ｐ＜００５）和ＨＡ，ＬＮ降低（Ｐ＜００１）有明显差异．结论三甲益肝冲剂对大鼠实验性肝纤维化有明显预防作用，其效果优于大黄庶虫虫丸．     

丙型肝炎抗体阳性的自身免疫性肝炎1例报告

正1病例资料患者女性,50岁,因"反复右胁部隐痛不适伴乏力1年余,加重1个月"入院。患者1年前无明显诱因下出现右胁部隐痛,伴乏力、腹胀、呃逆,呈进行性加重,自行服用中草药"鱼腥草",症状未见好转,后出现身目黄染,小便洗肉水样,至当地人民医院就诊,期间查"ALT900 U/L",诊断为"慢性丙型肝炎"。服用"多烯磷脂酰胆碱、复方甘草酸苷、双环醇、葡醛内酯、复合维生素B",病情控制欠佳。1年内反复5次转氨酶升     

Juvenile autoimmune hepatitis: Spectrum of the disease

Juvenile autoimmune hepatitis(JAIH) is a progressive inflammatory liver disease, affecting mainly young girls, from infancy to late adolescence, characterized by active liver damage, as shown by high serum activity of aminotransferases, by elevated immunoglobulin G levels, high titers of serum non organ-specific andorgan-specific autoantibodies, and by interface hepatitis on liver biopsy. It is a multifactorial disease of unknown etiology in which environmental factors act as a trigger in genetically predisposed individuals. Two types of JAIH are identified according to the autoan-tibody panel detected at diagnosis: AIH-1, characterized by the presence of anti-smooth muscle antibody and/or antinuclear antibody and AIH-2, by anti-liver-kidney microsomal antibody type 1 and/or by the presence of anti-liver cytosol type 1 antibody. Epidemiological distribution, genetic markers, clinical presentation and pattern of serum cytokines differentiate the two types of AIH suggesting possible pathogenetic mechanisms. The most effective therapy for AIH is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis. The aim of this review is to outline the most significant and peculiar features of JAIH, based largely on our own personal database and on a review of current literature.     

自身免疫性肝炎的肝组织病理学诊断

自身免疫性肝炎的肝组织病理学可表现各异,界面性肝炎伴淋巴-浆细胞浸润、“玫瑰花环”样结构及穿入现象是相对特征性表现,但非特异性。以上组织学表现需结合临床资料排除其他原因引起的肝炎才能诊断。主要阐述了自身免疫性肝炎的特征性组织学表现和特殊类型自身免疫性肝炎的组织学表现,讨论了组织学在自身免疫性肝炎的诊断与鉴别诊断中的价值。     

Prognostic implications of antibodies to Ro/SSA and soluble liver antigen in type 1 autoimmune hepatitis

Background: Antibodies to soluble liver antigen are frequently co‐expressed with antibodies to ribonucleoprotein/Sjögren's syndrome A (Ro/SSA) in autoimmune hepatitis. Aims: Our goals were to evaluate the prognostic implications of antibodies to Ro/SSA in type 1 autoimmune hepatitis and to determine their independence from antibodies to soluble liver antigen. Methods: Three hundred and seventy‐six serum samples from 170 patients were tested by enzyme immunoassays. Results: Sixty‐five patients (38%) had antibodies to Ro52; 11 patients (6%) had antibodies to Ro60; and 27 patients had antibodies to soluble liver antigen (16%). Twenty‐six patients with antibodies to Ro52 had antibodies to soluble liver antigen (40%), and 26 patients with antibodies to soluble liver antigen had antibodies to Ro52 (96%). Patients with antibodies to Ro52 and antibodies to soluble liver antigen had a higher frequency of human leucocyte antigen (HLA) DRB1^*03 (78 vs 50%, P=0.05) and lower occurrence of HLA DRB1^*04 (22 vs 57%, P=0.01) than patients with antibodies to Ro52 alone. Antibodies to Ro52 alone [hazard ratio (HR), 2.90; 95% confidence interval (CI), 1.18–7.14, P=0.02] and antibodies to Ro52 in conjunction with antibodies to soluble liver antigen (HR, 2.98; 95% CI, 1.07–8.43, P=0.04) were independently associated with the development of cirrhosis and hepatic death or liver transplantation. Conclusions: Antibodies to Ro52 alone and antibodies to Ro52 in conjunction with antibodies to soluble liver antigen are independently associated with a poor prognosis in type 1 autoimmune hepatitis. The prognostic implications ascribed to antibodies to soluble liver antigen may reflect their almost invariable concurrence with antibodies to Ro52.     

Autoimmune hepatitis: East meets west

Autoimmune hepatitis (AIH) is an inflammatory liver disease with diverse clinical spectrum, which predominantly affects females. This review provides detailed comparisons of epidemiology, genetic predispositions, clinical features, risk factors of hepatocellular carcinoma, and mortality in AIH patients between eastern and western countries. AIH prevalence and incidence are lower in Asia‐Pacific area than in Europe and America. European and American patients seem to have more severe disease, characterized with human leukocyte antigen‐DR3 haplotype, younger age, more AIH‐induced “cirrhosis” at diagnosis, higher elevated serum immunoglobulin G levels, and positive rate of antisoluble liver antigen/liver pancreatitis. The overall AIH diagnostic accuracy of revised original criteria and simplified scoring system are similar in European/American populations and Asian. Cirrhosis at presentation and non‐response to immunosuppressive therapy within 1 year are the most important predictors for poor prognosis of AIH patients.