简体中文

AC-9-核仁斑片型

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描述
核仁和卡哈尔体常出现不规则或斑块状染色，常伴有中期板染色体周围区域的染色。
Irregular/clumpy staining of the nucleoli and Cajal bodies often accompanied by peri-chromosomal staining of the metaphase plates.
抗原相关性 纤维蛋白（U3RNA小核仁核糖核蛋白复合物中的主要蛋白质） fibrillarin (a major protein in the U3RNA small nucleolar ribonucleoprotein complex).

  • 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

  • ►可见于系统性硬化症患者(48)。

    ►若临床疑似系统性硬化症，推荐后续检测抗U3RNP/核纤蛋白（fibrillarin）抗体。该抗原包含在疾病特异性免疫检测试剂中（例如，系统性硬化症谱*）(48)。

    ►若经免疫检测法确认有抗U3RNP/核纤蛋白（fibrillarin）抗体反应性，则其与弥散性系统性硬化症临床相关，且提示更易罹患肺动脉高压、骨骼肌疾病、严重心脏受累、胃肠动力障碍等(23,48-50)。

    ▶在系统性硬化症患者中，抗U3RNP/核纤蛋白（fibrillarin）抗体在非裔美国人、拉丁裔美国人中最为常见(48,49,51)。

    注：尽管已有商品化的抗U3RNP/核纤蛋白（fibrillarin）抗体免疫检测试剂，但其低敏感性等技术问题应纳入考量(24)。

    *炎症性肌病谱、系统性硬化症谱、自身免疫性肝病谱（扩展版）等检测试剂可能仅供临床专业实验室使用。



    ▶Found in patients with SSc (48)
    ▶If SSc is clinically suspected, it is recommended to perform a follow-up test for anti-U3RNP/fibrillarin antibodies; the antigen is included in disease specific immunoassays (i.e., SSc profile*) (48)
    ▶If confirmed as anti-U3RNP/fibrillarin reactivity by immunoassay, the clinical association is with diffuse SSc, increased incidence of pulmonary arterial hypertension, skeletal muscle disease, severe cardiac involvement, and gastrointestinal dysmotility (23, 48–50)

    ▶Among SSc patients, anti-U3RNP/fibrillarin antibodies are most commonly found in African American and Latin American patients (48, 49, 51)

    Notes: Although some anti-U3RNP/fibrillarin immunoassays are commercially available, technical issues relating to the limited sensitivity of these immunoassays should be taken into consideration (24).

    *Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.

  • 二级信息
    Second level information
  • 无

    None


  • 参考文献
  • 23.Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358-67.
    24.Mehra S, Walker J, Patterson K, et al. Autoantibodies in systemic sclerosis. Autoimmun Rev 2013;12:340-54.
    48.Okano Y, Steen VD, Medsger TA. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992;35:95-100.
    49.Arnett FC, Reveille JD, Goldstein R, et al. Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis. Arthritis Rheum 1996;39:1151-60.
    50.Tormey VJ, Bunn CC, Denton CP, et al. Anti-fibrillarin antibodies in systemic sclerosis. Rheumatology 2001;40:1157-62.
    51.Nandiwada SL, Peterson LK, Mayes MD, et al. Ethnic differences in autoantibody diversity and hierarchy: More clues from a US cohort of patients with systemic sclerosis. J Rheumatol 2016;43:1816-24.

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