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| Previous Nomenclature | None |
| Description |
Enhanced decoration of short segments of stress fibers with periodic dense bodies.
There is uncertainty in the characterization of sera with the AC-17 pattern. The composite figure shows the staining of two human sera (#1 and #2) each with two different HEp-2 brands. For serum #1, the distinctive staining illustrating AC-17 is much more prominent shown in brand #2 compared to brand #1. Similarly, serum #2 shows much more distinctive AC-17 staining in brand #3 while staining is not visible in brand #1. This limited data suggest that HEp-2 brand #1 is not quite sensitive for the detection of AC-17 as compared to brand #2 and #3. [One may question whether the Serum #2 staining with HEp-2 brand #3 is similar to some features of the cytoplasmic rods/rings staining (AC-23); however, this can be ruled out readily as HEp-2 brand #1 is known to show consistent AC-23 staining.] In summary, the ability to detect AC-17 may depend in part on staining on certain HEp-2 brands. |
| Antigen Association | alpha-actinin, vinculin |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ Found very infrequently in a routine diagnostic laboratory setting. ▶ Specific immunoassays for these autoantibodies are currently not commercially available. |
| Second level information |
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Autoantibodies to α-actinin: ▶ In systemic lupus erythematosus and lupus nephritis cross-reactive α-actinin and dsDNA autoantibodies have been reported as pathogenic [1]. ▶ Reported as part of the cell membrane antibody spectrum that characterizes patients with lupus nephritis; also reported as a biomarker that differentiated patients with lupus nephritis from those without renal involvement [2, 3]. ▶ Reported with relatively high prevalence (~40%) in patients with autoimmune hepatitis type 1 and associated with more severe disease, clinical and histological disease activity, predictor of therapeutic response, and double positivity with ssDNA antibodies [4-6]. Autoantibodies to vinculin: ▶ Reported in 2 of 31 patients with chronic inflammatory demyelinating neuropathy [7]. ▶ Reported in systemic sclerosis where they are associated with interstitial lung disease and gastrointestinal involvement [8-10]. ▶ Reported in patients with podocytopathies and idiopathic nephrotic syndrome to have pathogenic potential; may be useful in monitoring disease activity [11]. ▶ Vinculin and Cytolethal Distending Toxin B (CdtB) antibodies are biomarkers in irritable bowel syndrome [12-14]. Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e. solid phase antigen-specific immunoassays) and rarely demonstrate correlations with the AC-17 pattern. |
| References |
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1. Zhao Z, Weinstein E, Tuzova M, Davidson A, Mundel P, Marambio P, Putterman C. Cross-reactivity of human lupus anti-DNA antibodies with alpha-actinin and nephritogenic potential. Arthritis Rheum. 2005;52:522-30. 2. Seret G, Canas F, Pougnet-Di Costanzo L, Hanrotel-Saliou C, Jousse-Joulin S, Le Meur Y, Saraux A, Valeri A, 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. 3. Zhang WH, Pan HF, Zhao XF, Ye DQ, Li XP, Xu JH. Anti-alpha-actinin antibodies in relation to new-onset systemic lupus erythematosus and lupus nephritis. Mol Biol Rep. 2010;37:1341-5. 4. Gueguen P, Dalekos G, Nousbaum JB, Zachou K, Putterman C, Youinou P, Renaudineau Y. Double reactivity against actin and alpha-actinin defines a severe form of autoimmune hepatitis type 1. J Clin Immunol. 2006;26:495-505. 5. Renaudineau Y, Dalekos GN, Gueguen P, Zachou K, Youinou P. Anti-alpha-actinin antibodies cross-react with anti-ssDNA antibodies in active autoimmune hepatitis. Clin Rev Allergy Immunol. 2008;34:321-5. 6. Zachou K, Oikonomou K, Renaudineau Y, Chauveau A, Gatselis N, Youinou P, Dalekos GN. Anti-alpha actinin antibodies as new predictors of response to treatment in autoimmune hepatitis type 1. Aliment Pharmacol Ther. 2012;35:116-25. 7. Beppu M, Sawai S, Satoh M, Mori M, Kazami T, Misawa S, Shibuya K, Ishibashi M, et al. Autoantibodies against vinculin in patients with chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 2015;287:9-15. 8. Ibrahim NH, Fawzy IM, Gouda TM, El Sayed RAH, Morsi MH, Sabry ASM, Hashaad NI. Anti-vinculin antibodies as a novel biomarker in Egyptian patients with systemic sclerosis. Clin Rheumatol. 2022;41:3401-9. 9. Adler BL, McMahan Z. Anti-vinculin autoantibodies in systemic sclerosis: a step toward a novel biomarker? Clin Rheumatol. 2021;40:809-11. 10. Herran M, Adler BL, Perin J, Morales W, Pimentel M, McMahan ZH. Antivinculin Antibodies in Systemic Sclerosis: Associations With Slow Gastric Transit and Extraintestinal Clinical Phenotype. Arthritis Care Res (Hoboken). 2023;75:2166-73. 11. Meng H, Wang D, Zheng C, Zhou C, Mao X, Gu J, Qiao X, Liu F, et al. Autoantibodies Targeting Vinculin Reveal Novel Insight into the Mechanisms of Autoimmune Podocytopathies. Research (Wash D C). 2025;8:0722. 12. Rezaie A, Park SC, Morales W, Marsh E, Lembo A, Kim JH, Weitsman S, Chua KS, et al. Assessment of Anti-vinculin and Anti-cytolethal Distending Toxin B Antibodies in Subtypes of Irritable Bowel Syndrome. Dig Dis Sci. 2017;62:1480-5. 13. Vasapolli R, Schulz C, Schweden M, Casen C, Kirubakaran GT, Kirste KH, Macke L, Link A, et al. Gut microbiota profiles and the role of anti-CdtB and anti-vinculin antibodies in patients with functional gastrointestinal disorders (FGID). Eur J Clin Invest. 2021;51:e13666. 14. Zaki MES, Elhammady D, Foda Salama M, Abdelsalam M, Osman AOB. Study of Antibodies to Cytolethal Distending Toxin B (CdtB) and Antibodies to Vinculin in Patients with Irritable Bowel Syndrome. F1000Res. 2021;10:303. |
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