独家 | 自己抗体（第三版）要点分享（连载七）：自己免疫性肝炎的自己抗体、抗组织转谷氨酰胺酶......-外链论坛

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独家 | 自己抗体（第三版）要点分享（连载七）：自己免疫性肝炎的自己抗体、抗组织转谷氨酰胺酶......

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《自己抗体》（第三版）
2014年，《Autoantibodies（自己抗体）》第三版正式出版，新版本仍以第1版书为框架，然则因为自己抗体行业的快速发展，内容进行了全面更新。风湿界在接下来几周中，将连载《自己抗体》（第三版）的章节要点（中英文对照）。敬请关注！<img src="https://mmbiz.qpic.cn/mmbiz_png/KC4lHgxBOkFv3zlGRxbVVezW0pHlQSJJWOdWfdTASb6LSiaKaL0sVibuncBzdDIeiaibIeAgzvQCok7oZ4G5Wqp3yg/640?wx_fmt=png&tp=webp&wxfrom=5&wx_lazy=1&wx_co=1" style="width: 50%; margin-bottom: 20px;">
作者：
Yehuda Shoenfeld（以色列特拉维夫大学）
Pier Luigi Meroni （意大利米兰大学）
M. Eric Gershwin （美国加利福尼亚大学）
译者：黄志坚
邮箱：64847775@qq.com
译者注：
1.因为国内尚未有中文版《自己抗体（第三版）》出版，自己摘录英文版中章节要点并翻译成中文与大众共享。自己英语水平有限，欢迎大众吐槽。
2.本连载仅供学习用，请勿用于商场目的。
3.原版书籍中大部分章节有要点总结，但并不是所有章节都有。
第53章  自己免疫性肝炎的自己抗体
• 针对准确诊断自己免疫性肝炎（AIH）以及将自己免疫性肝炎（AIH）适当区分为其两个重点亚型，靠谱和快速检测自己抗体血清阳性是至关重要的。

• 近期，基于运用纯化或重组抗原的分子测定弥补了间接免疫荧光的常规自己抗体检测，但间接免疫荧光仍然是自己免疫血清学的支柱。尽管抗LKM-1和抗LC-1已然被确定为II型自己免疫性肝炎的特定分子靶点，然则用于鉴定II型自己免疫性肝炎的抗核抗体（ANA）和抗平滑肌抗体（SMA）需要更好的定义。
• 尽管自己抗体检测在自身免疫性肝炎中拥有诊断和预后功效，但每种抗体对肝损害的致病功效和潜在贡献仍然是有待进一步科研的课题。
CHAPTER 53 Autoantibodies in Autoimmune Hepatitis
• Reliable and prompt detection of autoantibody seropositivity is critical for the accurate diagnosis of AIH and the appropriate differentiation of AIH into its two main subtypes.
• Molecular assays based on the use of purified or recombinant antigen have recently complemented routine autoantibody testing by indirect IFL, which, however, remains the autoimmune serology mainstay. Although the specific molecular targets of anti-LKM-1 and anti-LC-1 in AIH-II have been clearly identified, those of ANA and SMA, characterizing AIH-II, require better definition.
• Despite the diagnostic and prognostic utility of autoantibody detection in AIH, the pathogenic role and potential contribution of each antibody to liver damage remains a topic for further research.
第54章  抗组织转谷氨酰胺酶和抗肌内膜抗体
• 大约0.5-1％的西欧和北美人群身患乳糜泻（CD），其中抗组织转谷氨酰胺酶抗体和抗肌内膜抗体（EMA）是重点的免疫学指标。

• 运用人提取或重组组织转谷氨酰胺酶的免疫测定法检测抗组织转谷氨酰胺酶IgA和IgG拥有高灵敏度和特异性水平(分别为91-99％和94-100％)。
• 用于鉴定抗组织转谷氨酰胺酶抗体的免疫测定办法的高灵敏度，这使得越来越多的乳糜泻病人能够被鉴定，这些病人一般拥有模糊或无症状的临床表现。
CHAPTER 54 Antitissue Transglutaminase and Antiendomysial Antibodies
• Approximately 0.5–1% of the western European and North American populations suffer from CD, for which anti-tTG antibodies and EMA are the main immunoserologic markers.
• Immunometric methods for the detection of anti-tTG IgA and IgG that use human extractive or recombinant tTG present high sensitivity and specificity levels (91–99% and 94–100%, respectively).
• The high sensitivity of immunometric methods used to identify anti-tTG antibodies has enabled an increasing number of patients with CD, often with vague or asymptomatic clinical presentations,to be identified.
第55章   抗醇溶蛋白和抗脱酰胺醇溶蛋白肽抗体
• 近年来，因为引入了更灵敏和特异的抗谷氨酰胺转氨酶实验，抗醇溶蛋白抗体（AGA）失去了许多诊断道理。
• 然而，近期运用由合成的脱酰胺醇溶蛋白肽（DGP）构成的涂层的ELISA测试表示出良好的诊断准确性，与抗转谷氨酰胺酶测试相当。
• 抗脱酰胺醇溶蛋白肽（DGP）抗体测定针对2-3岁以下病人的乳糜泻（CD）诊断尤其有用，由于抗转谷氨酰胺酶抗体可能在很强年龄显现。
• 针对IgA缺乏症病人，脱酰胺醇溶蛋白肽（DGP）IgG 的阳性亦很重要，由于它可能是独一的阳性血清学标志物（与抗组织转谷氨酰胺酶 IgG关联）。
• 在无抗肌内膜抗体，而抗醇溶蛋白IgG和IgA抗体阳性的状况下，抗转谷氨酰胺酶IgA、脱酰胺醇溶蛋白肽IgA或IgG可用作病人可能对非乳糜泻麸质敏锐症（GS）的指标。病人的肠或肠外症状提示其身患麸质关联疾患。
CHAPTER 55 Antigliadin and Antideamidated Gliadin Peptide Antibodies
• AGA have lost much of their diagnostic significance in recent years due to the introduction of the more sensitive and specific anti transglutaminase test.
• However, recent ELISA tests that use a coating consisting of synthetic deamidated gliadin peptides (DGP) show good diagnostic accuracy, comparable to those of the anti transglutaminase test.
• Anti-DGP antibody determination is especially useful for CD diagnosis in patients under 2–3 years old, as anti transglutaminase antibodies may appear at an older age.
• Positivity for IgG DGP can also be important in patients with IgA deficiency, in whom it may be the only positive serologic marker (in association with anti-tTG IgG).
• Positivity for IgG and IgA-type AGA, in the absence of EMA, IgA anti transglutaminase, IgA, or IgG DGP can be used as an indicator of possible nonceliac GS in patients with intestinal or extraintestinal symptoms suggestive of this gluten-related disorder.
第56章  肝胞浆1型抗原（LC-1）自己抗体，肝肾微粒体（LKM）自己抗体和肝微粒体（LM）自己抗体
• 在啮齿动物冷浴肝肾切片上，经过间接免疫荧光检测肝肾微粒体（LKM）自己抗体。这用作筛查工具。
• 需要运用重组抗原进行亚归类，以确定微粒体自己抗体的特异性。
• 内质网（ER）的药品代谢酶是疾患特异性B细胞反应性的重点目的。
• 经过免疫扩散和与甲酰转移酶环化脱氨酶的反应性检测肝胞浆1型抗原（LC-1）自己抗体。间接免疫荧光法并不靠谱。
• 与抗微粒体抗体关联的疾患包含药品诱导的肝炎、病毒性肝炎、自己免疫性肝炎（AIH）和1型自己免疫性多腺体综合症（APECED，APS-1）。
• 肝肾微粒体（LKM）和肝胞浆1型抗原（LC-1）抗体是自己免疫性肝炎的部分常规诊断性抗体。LKM-3和LKM-2和肝微粒体（LM）自己抗体是有用的诊断工具。
CHAPTER 56 Liver Cytosol Antigen Type 1 Autoantibodies (LC-1), Liver Kidney Microsomal Autoantibodies (LKM), and Liver Microsomal Autoantibodies (LM)
• Liver kidney microsomal (LKM) autoantibodies are detected by indirect immunofluorescence on rodent cryostat liver and kidney sections, which are employed as screening tools.
• Subclassification using recombinant antigens is required to determine the specificity of microsomal autoantibodies.
• Drug-metabolizing enzymes of the ER are major targets of disease-specific B-cell reactivities.
• LC-1 autoantibodies are detected by immunodiffusion and reactivity with formiminotransferase cyclodeaminase and not reliably by indirect immunofluorescence.
• Disease associations of antimicrosomal antibodies include drug-induced hepatitis, viral hepatitis, AIH, and the autoimmune polyglandular syndrome type 1 (APECED, APS-1).
• LKM and LC1 antibodies are part of the conventional repertoire of diagnostic antibodies for AIH, LKM-3, and LKM-2, and LM autoantibodies are useful diagnostic tools.
第57章  抗线粒体抗体
• 血清中的抗线粒体抗体（AMA）是原发性胆汁性肝硬化的标志。经过间接免疫荧光发掘90-95％的病例中存在这种抗体。

• 抗线粒体抗体的靶抗原已被鉴定为在糖酵解和氨基酸代谢中起功效的复合物。
• 抗线粒体抗体的致病功效仍然未知，由于抗线粒体抗体阴性病例无区别的疾患表型。
• 检测抗线粒体抗体的常规办法是在大鼠肾、胃和肝组织上进行间接免疫荧光，而基于更特异的重组抗原的其他技术的运用仅限于选定的病例。
• 缺乏血清抗线粒体抗体的原发性胆汁性肝硬化被叫作为自己免疫性胆管炎。
CHAPTER 57 Antimitochondrial Antibodies
• Serum AMA are the hallmark of PBC, being found at IIF in 90–95% of cases.
• AMA antigens have been identified as complexes active in the glycolytic and amino acid metabolism.
• The pathogenetic role of AMA remains unknown, as AMA-negative cases do not have a different disease phenotype.
• The routine method for AMA detection is IIF on rat kidney, stomach, and liver tissues, while the use of other techniques based on the more specific recombinant antigens is limited to selected cases.
• Proven PBC lacking serum AMA is called autoimmune cholangitis.
第58章  平滑肌自己抗体
• 平滑肌自己抗体（SMA）表率了针对区别细胞骨架成份的大范围自己反应，其中肌动蛋白是科研最多且临床关联的成份。
• 大鼠组织的间接免疫荧光仍然是检测平滑肌自己抗体和鉴定抗肌动蛋白特异性的最敏锐和特异的技术; 然而，运用丝状肌动蛋白的有前途且靠谱的酶联免疫吸附法（ELISA）测定正在研发中。
• 平滑肌自己抗体在自己免疫性肝炎（AIH）的诊断中拥有关联的诊断功效，但缺乏预后道理。相反，在乳糜泻中，拥有抗肌动蛋白特异性的IgA类平滑肌自己抗体与严重的粘膜损害关联，并可能有助于监测对无麸质膳食的反应。
CHAPTER 58 Smooth Muscle Autoantibodies
• SMA represent a large spectrum of autoreactivities targeting different cytoskeleton components, of which actin is the most studied and clinically relevant.
• IIF on rat tissues still represents the most sensitive and specific technique to detect SMA and identify antiactin specificity; however, promising and reliable ELISA assays with filamentous actin are being developed.
• SMA have a relevant diagnostic role in the diagnosis of AIH but are devoid of prognostic significance.In contrast, in celiac disease SMA of IgA class with antiactin specificity correlates with severe mucosal damage and may be helpful in monitoring the response to gluten-free diet.
第59章 凝血因子自己抗体
• 因为抗凝血因子自己抗体导致的流血性疾患很少见；然而，其中，最平常的是抗凝血因子VIII自己抗体。由自己抗体导致血管性血友病因子和因子XIII的缺陷是突发状况。其他凝血因子的抗体是非常罕见的，仅在零散的报告中描述。
• 在身患个人和家族流血史阴性的病人流血时，应怀疑潜在的凝血因子自己抗体，并最后经过特定的实验室检测确认。
• 确认临床可疑凝血因子自己抗体的诊断测试是血液凝血酶原时间（PT），活化部分凝血活酶时间（aPTT），混合科研和特定测定。
• 一旦确认凝血因子自己抗体的存在，治疗的目的应该是在流血的状况下替换或克服缺陷因子，消除自己抗体并控制其生成。
CHAPTER 59 Coagulation Factor Autoantibodies
• Bleeding disorders due to autoantibodies against coagulation factors are rare; however, among them, the most frequent are related to antifactor VIII autoantibodies. Acquired deficiencies of von Willebrand factor and factor XIII due to autoantibodies are emerging conditions. Antibodies to other coagulation factors are very rare and described only in scattered reports.
• In the presence of bleeding in patients with a negative personal and family hemorrhagic history, an underlying coagulation factor autoantibody should be suspected and eventually confirmed by specific laboratory tests.
• The diagnostic tests to confirm the clinical suspicion of coagulation factor autoantibody are PT, aPTT, mixing studies, and specific assays.
• Once the presence of coagulation factor autoantibodies has been confirmed, the therapy should be aimed at the replacement or overcoming of the deficient factor in case of bleeding and at the elimination of the autoantibody as well as the suppression of its production.
周2、周四
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独家 | 自己抗体（第三版）要点分享（连载七）：自己免疫性肝炎的自己抗体、抗组织转谷氨酰胺酶......