Coarse granular filamentous staining extending throughout the cytoplasm. e.g. anti-mitochondrial antibodies.
PDC-E2/M2, BCOADC-E2, OGDC-E2, E1? subunit of PDC, E3BP/protein X
common in PBC, SSc, rare in other SARD
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Commonly found in PBC, but also detected in SSc, including PBC-SSc overlap syndrome and PBC-SjS overlap syndrome
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
Additional antigens recognized include the E1? and E1? 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*)
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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.
Skare TL, Nisihara RM, Haider O, et al. Liver autoantibodies in patients with scleroderma. Clin Rheumatol 2011;30:129–32.
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.
Nardi N, Brito-Zero?n P, Ramos-Casals M, et al. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjo?gren’s syndrome: prevalence and clinical significance in 335 patients. Clin Rheumatol 2006;25:341–6.