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| Previous Nomenclature | Mitochondria-like |
| Description | Coarse granular filamentous staining extending from the nuclear envelope throughout the cytoplasm |
| Antigen Association | 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 |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ 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. |
| First level information references |
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| Second level information |
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▶ None |
| Second level information references |
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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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