Countable discrete nuclear speckles (6 to 20 nuclear dots/cell). e.g. SP-100.
Sp-100, PML proteins, MJ/NXP-2.
PBC, SARD, dermatomyositis.
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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).
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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
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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.