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| Previous Nomenclature | 1 to 6 nuclear dots, coiled body, Cajal bodies |
| Description | Countable discrete speckles (1 to 6 nuclear dots/cell in most cells). These are known as Cajal bodies or coiled bodies. e.g. anti-p80-coilin |
| Antigen Association | p80-coilin, SMN |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ The AC-7 pattern has low positive predictive value for any disease (40, 41) ▶ Antigens primarily localized in the dots include p80-coilin and SMN complex; specific immunoassays for these autoantibodies are currently not commercially available (42, 43) |
| First level information references |
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40. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun 1999;13:225-32. 41. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916-20. 42. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80-coilin. J Exp Med 1991;173:1407-19. 43. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972-8. |
| Second level information |
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▶ Anti‐p80‐coilin antibodies may rarely occur in SLE, (localized linear) SSc, and SjS (15‐17) ▶ Isolated (without anti‐snRNPs) anti‐SMN antibodies are reported in patients with AIM or SSc‐AIM overlap syndrome (18) ▶ The specificity of antibodies to p80‐coilin and the SMN complex can be confirmed by Western blot, solid phase immunoassays using recombinant proteins and immunoprecipitation (15, 18, 19) Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e. antigen‐specific immunoassays) and do not actually show clear correlations with the AC‐7 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available. |
| Second level information references |
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15. Andrade LE, Chan EK, Raska I, et al. Human autoantibody to a novel protein of the nuclear coiled body: immunological characterization and cDNA cloning of p80-coilin. J Exp Med 1991;173:1407-19. 16. Fujimoto M, Kikuchi K, Tamaki T, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol 1997;137:916-20. 17. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun 1999;13:225-32. 18. Satoh M, Chan JY, Ross SJ, et al. Autoantibodies to survival of motor neuron (SMN) complex in patients with polymyositis - immunoprecipitation of D-EF-G without other components of small nuclear ribonucleoproteins. Arthritis Rheum 2011;63:1972-8. 19. Amlani A, Hazlewood GS, Hamilton L, et al. Autoantibodies to the survival of motor neuron complex in a patient with necrotizing autoimmune myopathy. Rheumatology 2018;57:199-200. |
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