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| Previous Nomenclature | mitochondria-like |
| Description | Coarse granular filamentous staining extending throughout the cytoplasm. e.g. anti-mitochondrial antibodies. |
| Antigen Association | PDC-E2/M2, BCOADC-E2, OGDC-E2, E1α subunit of PDC, E3BP/protein X |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ 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. |
| First level information references |
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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. |
| Second level information |
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None |
| Second level information references |
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None
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