►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.