| 简体中文 | English | |
| 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 |
►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.
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.
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