▶ AC-10核型可见于多种疾病状态,包括系统性硬化症(SSc)、雷诺现象、干燥病(SjD)及恶性肿瘤[1-7]。
| 简体中文 | ||
| 同义词 | 核仁斑点状 | Nucleolar speckled |
| 描述 | 间期细胞:核仁内呈现密集分布但彼此清晰可辨的颗粒状荧光。中期细胞:在染色质团块中,可见多达5对明亮的核仁组成体(NOR)。有丝分裂期细胞:胞质可能呈弱阳性反应。 |
Densely distributed but distinct speckles in the nucleoli of interphase cells. In metaphase cells, up to 5 bright pairs of the nucleolar organizer region (NOR) can be seen as speckles in the chromatin mass. The cytoplasm of mitotic cells may be slightly positive |
| 抗原相关性 | RNA聚合酶I,NOR-90/hUBF | RNA polymerase I (RNApol-I), NOR90/hUBF |
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▶ 若观察到AC-10核型,应考虑后续检测NOR90抗体;该抗原已包含于疾病特异性免疫检测(如系统性硬化症谱系检测)之中[3, 4]。▶ NOR90抗体与副肿瘤性系统性硬化症(SSc)相关,同时也与SSc患者及特发性间质性肺炎患者的间质性肺病相关[8, 9]。▶ 虽然AC-10核型可见于含RNA聚合酶III抗体的血清中,但这被认为是由于与RNA聚合酶I和/或RNA聚合酶II抗体的交叉反应性所致。RNA聚合酶III抗体也与AC-4与AC-5混合核型相关[10];因此,若临床上怀疑SSc,建议进行RNA聚合酶III抗体的后续检测;目前尚无商业化的RNA聚合酶I或RNA聚合酶II抗体的特异性免疫检测试剂盒可供使用[1, 2]。▶The AC-10 pattern can be seen in various conditions, including systemic sclerosis (SSc), Raynaud’s phenomenon, Sjögren’s disease (SjD), and cancer [1-7]▶If the AC-10 pattern is observed, follow-up testing for NOR90 antibodies is to be considered; the antigen is included in disease specific immunoassays (i.e. SSc profile) [3, 4]▶NOR90 antibody associated with paraneoplastic SSc and with interstitial lung disease in SSc and idiopathic interstitial pneumonia patients [8, 9]▶While AC-10 is seen in sera with RNApol-III antibodies, this is thought to be due to concomitant reactivity with RNApol-I and/or RNApol-II antibodies. RNApol-III antibodies are also associated with mixed AC-4 and AC-5 patterns [10]; therefore, if SSc is clinically suspected, it is recommended to perform a follow-up test for RNApol-III antibodies; specific immunoassays for RNApol-I or RNApol-II antibodies are currently not commercially available [1, 2]
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二级信息Second level information
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参考文献
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1.Reimer G, Rose KM, Scheer U, Tan EM. Autoantibody to RNA polymerase I in scleroderma sera. J Clin Invest. 1987;79:65-722.Kuwana M, Kaburaki J, Mimori T, Tojo T, Homma M. Autoantibody reactive with three classes of RNA polymerases in sera from patients with systemic sclerosis. J Clin Invest. 1993;91:1399-4043.Fritzler MJ, von Muhlen CA, Toffoli SM, Staub HL, Laxer RM. Autoantibodies to the nucleolar organizer antigen NOR-90 in children with systemic rheumatic diseases. J Rheumatol. 1995;22:521-44.Fujii T, Mimori T, Akizuki M. Detection of autoantibodies to nucleolar transcription factor NOR 90/hUBF in sera of patients with rheumatic diseases, by recombinant autoantigen-based assays. Arthritis Rheum. 1996;39:1313-85.Imai H, Ochs RL, Kiyosawa K, Furuta S, Nakamura RM, Tan EM. Nucleolar antigens and autoantibodies in hepatocellular carcinoma and other malignancies. Am J Pathol. 1992;140:859-706.Lin W, Jia C, Yin H, Lu L. Clinical significance of the anti-Nucleolar Organizer Region 90 antibodies (NOR90) in systemic sclerosis. Eur J Intern Med. 2025;131:159-617.Dima A, Vonk MC, Garaiman A, Kersten BE, Becvar R, Tomcik M, Hoffmann-Vold AM, Castellvi I, et al. Clinical significance of the anti-Nucleolar Organizer Region 90 antibodies (NOR90) in systemic sclerosis: Analysis of the European Scleroderma Trials and Research (EUSTAR) cohort and a systematic literature review. Eur J Intern Med. 2024;125:104-108.Duffau P, Dimicoli S, Gensous N, Truchetet ME, Bordes C. Anti-NOR90 antibody associated with paraneoplastic systemic sclerosis. Clin Exp Rheumatol. 2022;40:2002-3
9.Yamashita Y, Yamano Y, Muro Y, Ogawa-Momohara M, Takeichi T, Kondoh Y, Akiyama M. Clinical significance of anti-NOR90 antibodies in systemic sclerosis and idiopathic interstitial pneumonia. Rheumatology (Oxford). 2022;61:1709-16
10.Parker JC, Burlingame RW, Webb TT, Bunn CC. Anti-RNA polymerase III antibodies in patients with systemic sclerosis detected by indirect immunofluorescence and ELISA. Rheumatology (Oxford). 2008;47:976-9
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