PORTUGUÊS / ENGLISH

AC-7 - Few Nuclear Dots

Descrição
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.
Associação Antigênica
p80-coilin, SMN
Doença Associada
SjS, SLE, SSc, PM, asymptomatic individuals.
Relevância Clínica (Primeiro Nível)
The AC-7 pattern has low positive predictive value for any disease.
Onouchi, Muro & Tomita, 1999Fujimoto, Kikuchi, Tamaki et al., 1997
Antigens primarily localized in the dots include p80-coilin and SMN complex; specific immunoassays for these autoantibodies are currently not commercially available.
Andrade, Chan, Raska et al., 1991Satoh, Chan, Ross et al., 2011
Relevância Clínica (Segundo Nível)
Anti-p80-coilin antibodies may rarely occur in SLE, (localized linear) SSc, and SjS.
Andrade, Chan, Raska et al., 1991Fujimoto, Kikuchi, Tamaki et al., 1997Onouchi, Muro & Tomita, 1999
Isolated (without anti-snRNPs) anti-SMN antibodies are reported in patients with AIM or SSc-AIM overlap syndrome.
Satoh, Chan, Ross et al., 2011
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.
Andrade, Chan, Raska et al., 1991Satoh, Chan, Ross et al., 2011Amlani, Hazlewood, Hamilton et al., 2018
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.
Damoiseaux, Andrade, Carballo et al., 2019
Referências
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.

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.

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.

Onouchi H, Muro Y, Tomita Y. Clinical features and IgG subclass distribution of anti-p80 coilin antibodies. J Autoimmun 1999;13:225-32.

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.