简体中文

AC-9-核仁斑片型

同义词 无 None
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
  • 1.Okano Y, Steen VD, Medsger TA, Jr. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum. 1992;35:95-100.
    2.Benyamine A, Bertin D, Resseguier N, Heim X, Bermudez J, Launay D, Dubucquoi S, Hij A, et al. Quantification of Antifibrillarin (anti-U3 RNP) Antibodies: A New Insight for Patients with Systemic Sclerosis. Diagnostics (Basel). 2021;11:1064.
    3.Satoh M, Ceribelli A, Hasegawa T, Tanaka S. Clinical Significance of Antinucleolar Antibodies: Biomarkers for Autoimmune Diseases, Malignancies, and others. Clin Rev Allergy Immunol. 2022;63:210-39.
    4.Benyamine A, Bertin D, Resseguier N, Heim X, Bermudez J, Launay D, Dubucquoi S, Hij A, et al. Quantification of Antifibrillarin (anti-U3 RNP) Antibodies: A New Insight for Patients with Systemic Sclerosis. Diagnostics (Basel). 2021;11.
    5.Arnett FC, Reveille JD, Goldstein R, Pollard KM, Leaird K, Smith EA, Leroy EC, Fritzler MJ. Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis. Arthritis Rheum. 1996;39:1151-60.
    6.Tormey VJ, Bunn CC, Denton CP, Black CM. Anti-fibrillarin antibodies in systemic sclerosis. Rheumatology (Oxford). 2001;40:1157-62.
    7.Johnson SR, Fransen J, Khanna D, Baron M, van den Hoogen F, Medsger TA, Jr., Peschken CA, Carreira PE, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res. 2012;64:358-67.
    8.Nandiwada SL, Peterson LK, Mayes MD, Jaskowski TD, Malmberg E, Assassi S, Satoh M, Tebo AE. 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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