简体中文

AC-21-胞浆网状/线粒体样型

同义词 线粒体样型 Mitochondria-like
描述 分裂间期细胞胞浆内呈现自核膜延伸至全胞浆的粗颗粒样荧光
Coarse granular filamentous staining extending from the nuclear envelope throughout the cytoplasm
抗原相关性
自身抗体主要针对丙酮酸脱氢酶（PDH）复合物的组分，尤以 E2 亚基（PDH-E2）为主要靶抗原。常见抗原包括：PDC-E2/M2、BCOADC-E2、OGDC-E2、PDC 的 E1α 亚基、E3BP / 蛋白 X
Autoantibodies are primarily directed to components of the pyruvate dehydrogenase (PDH) complex, and in particular the E2-subunit (PDH-E2). The common antigens are: PDC-E2/M2, BCOADC-E2, OGDC-E2, E1α subunit of PDC, E3BP/protein X

  • 临床相关性

    一级信息

    关于临床相关性和缩写列表

    Clinical Relevance

    First level information

    About Clinical Relevance & List of Abbreviations

  • ▶ 常见于原发性胆汁性胆管炎（PBC），但也可在系统性硬化症（SSc）中检出，包括 PBC-SSc 重叠综合征、PBC - 干燥综合征（SjD）重叠综合征，以及其他系统性自身免疫性风湿病 [1-7]。
    ▶ 如临床上疑似 PBC，建议进行 AMA 的后续检测；AMA 历来通过 IFA 法在啮齿类动物组织（肝、胃、肾）上检出，也可采用疾病特异性免疫检测试剂（即肝病谱）以及部分常规多抗原阵列进行检测。


    ▶Commonly found in primary biliary cholangitis (PBC), but also detected in systemic sclerosis (SSc), including PBC-SSc overlap syndrome and PBC-Sjögren’s disease (SjD) overlap syndrome, and in other systemic autoimmune rheumatic diseases [1-7].
    ▶If PBC is clinically suspected, a follow-up test for AMA is recommended, historically detected by IFA on rodent tissue (liver, stomach, kidney) and using disease specific immunoassays (i.e., liver profile) as well as in some routine multianalyte arrays.

  • 二级信息
    Second level information

  • ▶无

    ▶None
  • 参考文献
  • 1.EASL Clinical Practice Guidelines on the management of autoimmune hepatitis. J Hepatol. 2025;83:453-501.
    2.Shuai Z, Wang J, Badamagunta M, Choi J, Yang G, Zhang W, Kenny TP, Guggenheim K, et al. The fingerprint of antimitochondrial antibodies and the etiology of primary biliary cholangitis. Hepatology. 2017;65:1670-82.
    3.Skare TL, Nisihara RM, Haider O, Azevedo PM, Utiyama SR. Liver autoantibodies in patients with scleroderma. Clin Rheumatol. 2011;30:129-32.
    4.Zheng B, Vincent C, Fritzler MJ, Senécal JL, Koenig M, Joyal F. Prevalence of Systemic Sclerosis in Primary Biliary Cholangitis Using the New ACR/EULAR Classification Criteria. J Rheumatol. 2017;44:33-9.
    5.Nardi N, Brito-Zeron P, Ramos-Casals M, Aguilo S, Cervera R, Ingelmo M, Font J. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjogren's syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol. 2006;25:341-6.
    6.Ceribelli A, Isailovic N, Gorlino C, Assandri R, Vecellio M, De Santis M, Satoh M, Selmi C. Antigen Reactivity and Clinical Significance of Autoantibodies Directed Against the Pyruvate Dehydrogenase Antigen Complex in Patients With Connective Tissue Disease. Front Immunol. 2022;13:822996.
    7.Bolis F, Cazzaniga G, Pagni F, Invernizzi P, Carbone M, Gerussi A. The phenotypic landscape of primary biliary cholangitis and autoimmune hepatitis variants. Gastroenterol Hepatol. 2025;48:502225.

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