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| Previous Nomenclature | 1 to 6 nuclear dots, coiled body, Cajal bodies. |
| Description | One to 6 nuclear discrete speckles/cell. The chromatin mass is not stained. These speckles (dots) are known as Cajal bodies, coiled bodies and nuclear gems or ‘gemini of coiled bodies’. |
| Antigen Association | p80 coilin, Survival Motor Neuron (SMN) complex (including SMN protein and other proteins known as gemins). |
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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 [1, 2] ▶ Antigens primarily localized in the dots include p80-coilin and proteins of the SMN complex; specific immunoassays for these autoantibodies are currently not commercially available [3, 4] |
| Second level information |
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▶ p80‐coilin antibodies may rarely occur in systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren’s disease SjD [1-3] ▶ Isolated (without anti‐snRNPs) anti-SMN is reported in patients with idiopathic inflammatory myopathies (IIM) or SSc‐IIM overlap syndrome [4, 5] ▶ The detection of both anti-p80-coilin (AC-7) and anti-DFS70 (AC-2) was reported in Japan [6] and it is also the experience in some reference laboratories in Europe. The implication is that AC-7 thus can be found in people without SARD as reported [6] ▶ 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 [3, 4, 7] Most reports describe autoantibodies directly binding antigen‐specific immunoassays and do not actually show clear correlations with the AC‐7 pattern |
| References |
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1. Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun. 1999;13:225-32 2. Fujimoto M, Kikuchi K, Tamaki T, Yazawa N, Kubo M, Ihn H, Sato S, Soma Y, et al. Distribution of anti-p80-coilin autoantibody in collagen diseases and various skin diseases. Br J Dermatol. 1997;137:916-20 3. Andrade LE, Chan EKL, Raska I, Peebles CL, Roos G, Tan EM. 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 4. Satoh M, Chan JY, Ross SJ, Ceribelli A, Cavazzana I, Franceschini F, Li Y, Reeves WH, 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 5. Landon-Cardinal O, Baril-Dionne A, Hoa S, Meyer A, Leclair V, Bourre-Tessier J, Mansour AM, Zarka F, et al. Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies. RMD Open. 2020;6 6. Goto N, Sugiura K, Ogawa Y, Watanabe A, Onouchi H, Tomita Y, Muro Y. Anti-p80 coilin autoantibodies react with a conserved epitope and are associated with anti-DFS70/LEDGF autoantibodies. J Autoimmun. 2006;26:42-51 7. Amlani A, Hazlewood GS, Hamilton L, Satoh M, Fritzler MJ. Autoantibodies to the survival of motor neuron complex in a patient with necrotizing autoimmune myopathy. Rheumatology (Oxford). 2018;57:199-200 |
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