简体中文

AC-8-核仁均质型

同义词 无 None
描述
整个核仁呈弥漫性荧光染色，而中期板未见着色。该模式常与细颗粒型核荧光染色（AC-4）合并出现。
Diffuse fluorescence of the entire nucleolus, while the metaphase plate shows no staining. Frequently seen in combination with a fine speckled nuclear staining (AC-4).
抗原相关性
PM/Scl-75、PM/Scl-100、Th/To、B23/核磷蛋白（nucleophosmin）、C23/核仁素（nucleolin）、No55/SC65 注释1. 抗SS-A/Ro60抗体在HEp-2000载片（IMMUNO CONCEPTS公司）上呈现核仁染色模式，与其他HEp-2载片上的表现不同。
 PM/Scl-75, PM/Scl-100, Th/To, B23/nucleophosmin, C23/nucleolin, No55/SC65 Note #1. Anti-SS-A/Ro60 shows nucleolar staining on HEp-2000 slides (IMMUNO CONCEPTS) different from other HEp-2 slides.

  • 临床相关性

    一级信息

    关于临床相关性和缩写列表

    Clinical Relevance

    First level information

    About Clinical Relevance & List of Abbreviations

  • ►该核型可见于系统性硬化症（SSc）、SSc-特发性炎症性肌病（IIM）重叠综合征患者，以及具有其他系统性自身免疫性风湿病（SARD）临床表现的患者[1,2]。

    ►若临床疑似局限性皮肤型SSc，建议行Th/To抗体后续检测（即SSc特异性抗体谱）[1,2]。

    ►若临床疑似SSc-IIM重叠综合征，建议行PM/Scl抗体反应性检测；一般而言，PM/Scl抗体除呈现AC-8核型外，还可产生弥漫性核细颗粒型荧光染色[3,4]。

    ►其他可识别的抗原包括B23/核磷蛋白、No55/SC65及C23/核仁素，但这些自身抗体的临床意义尚未充分确立；目前尚无商品化的特异性免疫检测试剂盒可供上述自身抗体的检测。

    Th/To为一大分子复合物，包含多种自身抗体靶抗原（hPOP1、Rpp25、Rpp38），各抗原对自身免疫病的敏感性与特异性各异。了解免疫检测中所采用的具体抗原成分至关重要[1,5]。

    ▶Found in patients with systemic sclerosis (SSc), SSc-idiopathic inflammatory myopathies (IIM) overlap, and patients with clinical manifestations of other systemic autoimmune rheumatic diseases (SARD) [1, 2]
    ▶If limited cutaneous SSc is clinically suspected, it is recommended to perform a follow-up test for Th/To antibodies (i.e., SSc profile) [1, 2]
    ▶If SSc-IIM overlap syndrome is clinically suspected, it is recommended to perform a follow-up test for PM/Scl antibody reactivity; in general, PM/Scl antibodies yield a diffuse nuclear fine speckled staining in addition to the AC-8 pattern [3, 4]
    ▶Other antigens recognized include B23/nucleophosmin, No55/SC65, and C23/nucleolin, but the clinical significance of these autoantibodies is not well established; specific immunoassays for these autoantibodies are currently not commercially available
    Th/To is a macromolecular complex comprising several autoantibody targets (hPOP1, Rpp25, Rpp38) with varying sensitivity and specificity for autoimmune disease. It is important to know which of these antigens are used in immunoassays [1, 5]
  • 二级信息
    Second level information

  • ►由抗Th/To反应性所致的AC-8核型亦可见于少数系统性红斑狼疮（SLE）、未分化结缔组织病（UCTD，即具有风湿症状但未达到SARD诊断标准的患者）、无皮肤硬化的SSc、特发性间质性肺病及肺动脉高压患者[6,7]。

    ►因抗PM/Scl反应性而呈现AC-8核型的自身抗体阳性患者，除具有IIM及SSc的临床特征外，还可能表现出SLE及干燥综合征（Sjögren's disease）的多种临床表现[3]。

    ▶The AC‐8 pattern resulting from the anti‐Th/To reactivity is also seen in rare patients with systemic lupus erythematosus (SLE), undifferentiated connective tissue disease (UCTD; i.e., patients with rheumatic symptoms not satisfying a SARD diagnosis), SSc sine scleroderma, idiopathic interstitial lung disease and pulmonary hypertension [6, 7]
    ▶Patients with autoantibodies revealing the AC‐8 pattern due to anti‐PM/Scl reactivity may have, in addition to the clinical features of IIM and SSc, various clinical manifestations of SLE and Sjögren’s disease [3]
  • 参考文献
  • 1.Mahler M, Fritzler MJ, Satoh M. Autoantibodies to the mitochondrial RNA processing (MRP) complex also known as Th/To autoantigen. Autoimmun Rev. 2015;14:254-7
    2.Ceribelli A, Cavazzana I, Franceschini F, Airo P, Tincani A, Cattaneo R, Pauley BA, Chan EKL, et al. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma. J Rheumatol. 2010;37:2071-5
    3.Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev. 2007;6:432-7
    4.D'Aoust J, Hudson M, Tatibouet S, Wick J, Canadian Scleroderma Research G, Mahler M, Baron M, Fritzler MJ. Clinical and serologic correlates of anti-PM/Scl antibodies in systemic sclerosis: a multicenter study of 763 patients. Arthritis Rheumatol. 2014;66:1608-15
    5.Mahler M, Gascon C, Patel S, Ceribelli A, Fritzler MJ, Swart A, Chan EKL, Satoh M. Rpp25 is a major target of autoantibodies to the Th/To complex as measured by a novel chemiluminescent assay. Arthritis Res Ther. 2013;15:R50
    6.Fischer A, Pfalzgraf FJ, Feghali-Bostwick CA, Wright TM, Curran-Everett D, West SG, Brown KK. Anti-th/to-positivity in a cohort of patients with idiopathic pulmonary fibrosis. J Rheumatol. 2006;33:1600-5
    7.Fischer A, Meehan RT, Feghali-Bostwick CA, West SG, Brown KK. Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. Chest. 2006;130:976-81

FAQ