简体中文

AC-21-胞浆网状/线粒体样型

同义词 线粒体样 mitochondria-like
描述

分裂间期细胞胞浆内呈现粗颗粒样荧光。

Coarse granular filamentous staining extending throughout the cytoplasm. e.g. anti-mitochondrial antibodies.
抗原相关性 PDC-E2/M2, BCOADC-E2, OGDC-E2, E1α PDC亚单位, E3BP/蛋白 X 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,但也可在系统性硬化症中检出,包括原发性胆汁性胆管炎-系统性硬化症重叠综合征、原发性胆汁性胆管炎-干燥综合征重叠综合征 (93-97)

    ►如临床上疑似为原发性胆汁性胆管炎,建议进行AMA的后续检测,该检测历来是用IIFA法在啮齿类动物组织 (肝、胃、肾)上进行;这些自身抗体主要针对PDH复合物,尤其是E2亚基 (PDH-E2);抗原包含于疾病特异性免疫检测试剂中 (即,肝病谱*)以及一些常规的ENA抗体谱中 (93,94)

    ►其余所识别的抗原包括PDH的E1α 和 E1β亚基、PDH的E3结合蛋白和2-OGDC;这些抗原仅包含于扩展的疾病特异性免疫检测试剂中 (即,扩展的肝病谱*) (93,94)

    *肌炎谱、系统性硬化症谱和 (扩展的) 肝病谱可能仅限于专业临床实验室应用。



    ► Commonly found in PBC, but also detected in SSc, including PBC-SSc overlap syndrome and PBC-SjS overlap syndrome (93–97)

    ► If PBC is clinically suspected it is recommended to perform a follow-up test for AMA, historically detected by IIFA on rodent tissue (liver, stomach, kidney); these autoantibodies are primarily directed to the PDH complex, and in particular the E2-subunit (PDH-E2); the antigen is included in disease specific immunoassays (i.e., liver profile*) as well as in some routine ENA profiles (93, 94)

    ► Additional antigens recognized include the E1α and E1β subunits of PDH, the E3-binding protein of PDH, and the 2-OGDC; these antigens are only included in extended disease specific immunoassays (i.e., extended liver profile*) (93, 94)

    *Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.

  • 一级信息参考文献
    First level information references

  • 93. European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017;67:145–72.

    94. Shuai Z, Wang J, Badamagunta M, et al. The fingerprint of antimitochondrial antibodies and the etiology of primary biliary cholangitis. Hepatology 2017;65:1670–82.

    95. Skare TL, Nisihara RM, Haider O, et al. Liver autoantibodies in patients with scleroderma. Clin Rheumatol 2011;30:129–32.

    96. Zheng B, Vincent C, Fritzler MJ, et al. Prevalence of systemic sclerosis in primary biliary cholangitis using the new ACR/EULAR classification criteria. J Rheumatol 2017;44:33–9.

    97. Nardi N, Brito-Zerón P, Ramos-Casals M, et al. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjögren’s syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol 2006;25:341–6.



    93. European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol 2017;67:145–72.

    94. Shuai Z, Wang J, Badamagunta M, et al. The fingerprint of antimitochondrial antibodies and the etiology of primary biliary cholangitis. Hepatology 2017;65:1670–82.

    95. Skare TL, Nisihara RM, Haider O, et al. Liver autoantibodies in patients with scleroderma. Clin Rheumatol 2011;30:129–32.

    96. Zheng B, Vincent C, Fritzler MJ, et al. Prevalence of systemic sclerosis in primary biliary cholangitis using the new ACR/EULAR classification criteria. J Rheumatol 2017;44:33–9.

    97. Nardi N, Brito-Zerón P, Ramos-Casals M, et al. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjögren’s syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol 2006;25:341–6.

  • 二级信息
    Second level information

  • None
  • 二级信息参考文献
    Second level information references

  • None

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