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| Previous Nomenclature | None |
| Description | Variable sized coarse speckles dispersed throughout the nucleoplasm. The nucleoli are typically not stained. The chromatin mass of mitotic cells (metaphase, anaphase, and telophase) is, in contrast to the cytoplasm surrounding the chromatin mass, not stained. |
| Antigen Association | Sp100, Sp140, NXP-2 |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ Found in a broad spectrum of autoimmune diseases, including primary biliary cholangitis (PBC), idiopathic inflammatory myopathies (IIM), dermatomyositis (DM), as well as other inflammatory conditions [1] ▶ If PBC is clinically suspected, follow-up tests for antibodies to Sp100 and Sp140 are recommended; in particular Sp100 antibodies are recommended as they are typically associated with PBC and have prognostic value; the Sp100 (and Sp140) antigen may be included in disease specific immunoassays (i.e., liver profile) [2-4] ▶ If DM is clinically suspected, it is recommended to perform a follow-up test for NXP-2 antibodies; these NXP-2 antibodies are highly specific for IIM, are found in up to one third of patients with juvenile DM, and have been reported to be associated with malignancies in adult IIM patients [5-7] |
| Second level information |
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None |
| References |
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1. Cozzani E, Drosera M, Riva S, Parodi A. Analysis of a multiple nuclear dots pattern in a large cohort of dermatological patients. Clin Lab. 2012;58:329-32 2. Granito A, Yang WH, Muratori L, Lim MJ, Nakajima A, Ferri S, Pappas G, Quarneti C, et al. PML nuclear body component Sp140 is a novel autoantigen in primary biliary cirrhosis. Am J Gastroenterol. 2010;105:125-31 3. Hu SL, Zhao FR, Hu Q, Chen WX. Meta-analysis assessment of GP210 and SP100 for the diagnosis of primary biliary cirrhosis. PLoS One. 2014;9:e101916 4. Rigopoulou EI, Bogdanos DP. Role of autoantibodies in the clinical management of primary biliary cholangitis. World J Gastroenterol. 2023;29:1795-810 5. Ceribelli A, Fredi M, Taraborelli M, Cavazzana I, Franceschini F, Quinzanini M, Tincani A, Ross SJ, et al. Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis. Arthritis Res Ther. 2012;14:R97 6. Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol. 2016;29:662-73 7. Ichimura Y, Matsushita T, Hamaguchi Y, Kaji K, Hasegawa M, Tanino Y, Inokoshi Y, Kawai K, et al. Anti-NXP2 autoantibodies in adult patients with idiopathic inflammatory myopathies: possible association with malignancy. Ann Rheum Dis. 2012;71:710-3 |
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