AC-6 - Multiple Nuclear Dots

Countable discrete nuclear speckles (6 to 20 nuclear dots/cell). e.g. SP-100.
Associação Antigênica
Sp-100, PML proteins, MJ/NXP-2.
Doença Associada
PBC, SARD, dermatomyositis.
Relevância Clínica (Primeiro Nível)
Found in a broad spectrum of autoimmune diseases, including PBC, AIM (DM), as well as other inflammatory conditions.
If PBC is clinically suspected, it is recommended to perform follow-up tests for anti-Sp100 (and PML/ Sp140) antibodies; in particular anti-Sp100 antibodies have the best clinical association with PBC and have added value, especially when associated with AMA; the Sp100 (and PML-Sp140) antigen is included in disease specific immunoassays (ie, liver profile).
If DM is clinically suspected, it is recommended to perform a follow-up test for anti-MJ/NXP-2 antibodies; these anti-MJ/NXP-2 antibodies are highly specific for AIM, are found in up to one third of patients with juvenile DM, and have been reported to be associated with malignancies in adult AIM patients; the antigen is included in disease specific immunoassays (i.e., inflammatory myopathy profile).
Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol 2016;29:662–73.

Ceribelli A, Fredi M, Taraborelli M, et al. Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis. Arthritis Res Ther

Conrad K, Scho?ssler W, Hiepe F. Autoantibodies in systemic autoimmune diseases: a diagnostic reference. 2. 3th edn. Autoantigens autoantibodies autoimmunity, 2015.

Cozzani E, Drosera M, Riva S, et al. Analysis of a multiple nuclear dots pattern in a large cohort of dermatological patients. Clin Lab 2012;58:329–32.

Granito A, Yang WH, Muratori L, et al. PML nuclear body component SP140 is a novel autoantigen in primary biliary cirrhosis. Am J Gastroenterol 2010;105:125–31.

Hu SL, Zhao FR, Hu Q, et al. Meta-analysis assessment of gp210 and Sp100 for the
diagnosis of primary biliary cirrhosis. PLoS One 2014;9:e101916.

Ichimura Y, Matsushita T, Hamaguchi Y, et al. Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with
malignancy. Ann Rheum Dis 2012;71:710–3.