| 简体中文 | ||
| 同义词 | 6-20个核点，NSpl，PML小体 | 6-20 nuclear dots, NSpI, PML bodies |
| 描述 | 出现清晰可数的点状荧光（通常6-20个），例如SP-100。 |
Countable discrete nuclear speckles (6 to 20 nuclear dots/cell). e.g. SP-100 |
| 抗原相关性 | Sp-100, PML 蛋白，MJ/NXP-2 | Sp-100, PML proteins, MJ/NXP-2 |
►广泛存在于多种自身免疫性疾病中，包括原发性胆汁性胆管炎、自身免疫性肌病（皮肌炎）以及其他炎症(34)
►对于临床疑似原发性胆汁性胆管炎的患者，建议后续检测抗Sp100（以及PML/Sp140）抗体；特别是抗Sp100抗体与原发性胆汁性胆管炎的临床相关性最佳，尤其是与AMA联合检测时，检测结果具有附加价值；Sp100（和PML/Sp140）抗原包含在疾病特异性免疫检测（例如，肝病谱*）中(17,35,36)
►对于临床疑似皮肌炎的患者，建议后续检测抗MJ/NXP-2抗体；抗MJ/NXP-2抗体对于自身免疫性肌病具有高度特异性，在多达三分之一的皮肌炎少年患者中发现，并且据报道与成人自身免疫性肌病患者的恶性肿瘤相关；该抗原包含在疾病特异性免疫检测中（例如，炎症性肌病谱*）(37-39)
*炎症性肌病谱、系统性硬化症谱和（扩展的）肝病谱的应用可能仅限于专业的临床实验室。
► Found in a broad spectrum of autoimmune diseases, including PBC, AIM (DM), as well as other inflammatory conditions (34)
► 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 (i.e., liver profile*) (17, 35, 36)
► 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*) (37–39)
*Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.
17. Conrad K, Schössler W, Hiepe F. Autoantibodies in organ specific autoimmune diseases. a diagnostic reference. 8. 2th edn. Autoantigens autoantibodies autoimmunity, 2017.
34. 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.
35. 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.
36. 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.
37. 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.
38. Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol 2016;29:662–73.
39. 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 2012;14.
17. Conrad K, Schössler W, Hiepe F. Autoantibodies in organ specific autoimmune diseases. a diagnostic reference. 8. 2th edn. Autoantigens autoantibodies autoimmunity, 2017.
34. 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.
35. 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.
36. 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.
37. 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.
38. Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol 2016;29:662–73.
39. 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 2012;14.