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| Previous Nomenclature | None |
| Description | Enhanced decoration of short segments, periodic dense bodies, along the stress fibers. e.g. anti-alpha-actinin, anti-vinculin. |
| Antigen Association | alpha-actinin, vinculin |
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Clinical Relevance
First level information About Clinical Relevance |
▶ Found very infrequently in a routine serology diagnostic setting ▶ Antigens recognized include α-actinin and vinculin; specific immunoassays for these autoantibodies are currently not commercially available |
| First level information references |
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| Second level information |
▶ Autoantibodies to α-actinin: ▶ In SLE and lupus nephritis cross-reactive anti-α-actinin and anti-dsDNA autoantibodies have been reported as pathogenic (21) ▶ Reported as part of the anti-cell membrane antibody spectrum that characterize patients with lupus nephritis; also reported as a biomarker to differentiate patients with lupus nephritis from those without renal involvement (22, 23) ▶ Reported with relatively high prevalence (~40%) in patients with AIH type 1 and associated with more severe disease, clinical and histological disease activity, predictor of therapeutic response, and double positivity with anti-ssDNA antibodies (24-26) ▶ Autoantibodies to vinculin: ▶ Reported in 2 of 31 patients with chronic inflammatory demyelinating neuropathy (27) Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e. antigen-specific immunoassays) and none actually shows correlations with the AC-17 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available. |
| Second level information references |
21. Zhao Z, Weinstein E, Tuzova M, et al. Cross-reactivity of human lupus anti-DNA antibodies with alpha-actinin and nephritogenic potential. Arthritis Rheum 2005;52:522-30. 22. Seret G, Canas F, Pougnet-Di Costanzo L, et al. Anti-alpha-actinin antibodies are part of the anti-cell membrane antibody spectrum that characterize patients with lupus nephritis. J Autoimmun 2015;61:54-61. 23. Zhang WH, Pan HF, Zhao XF, et al. Anti-alpha-actinin antibodies in relation to new-onset systemic lupus erythematosus and lupus nephritis. Mol Biol Rep 2010;37:1341-5. 24. Gueguen P, Dalekos G, Nousbaum JB, et al. Double reactivity against actin and alpha-actinin defines a severe form of autoimmune hepatitis type 1. J Clin Immunol 2006;26:495-505. 25. Renaudineau Y, Dalekos GN, Gueguen P, et al. Anti-alpha-actinin antibodies cross-react with anti-ssDNA antibodies in active autoimmune hepatitis. Clin Rev Allergy Immunol 2008);34:321-5. 26. Zachou K, Oikonomou K, Renaudineau Y, et al. Anti-alpha actinin antibodies as new predictors of response to treatment in autoimmune hepatitis type 1. Aliment Pharmacol Ther 2012;35:116-25. 27. Beppu M, Sawai S, Satoh M, et al. Autoantibodies against vinculin in patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol 2015;287:9-15. |
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