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AC-9-核仁斑片型

同义词 中心粒 None
描述

核仁和卡哈尔体呈不规则荧光,分裂期细胞表现为染色体周围的环状荧光。如:抗纤维蛋白抗体。

Irregular staining of the nucleoli and Cajal bodies with a peri-chromosomal staining at the metaphase plates. e.g. anti-fibrillarin.
抗原相关性 U3-snoRNP / 纤维蛋白 U3-snoRNP/fibrillarin
  • 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.

  • 一级信息参考文献
    First level information references

  • 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.



    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.

  • 二级信息
    Second level information
  • None


  • 二级信息参考文献
    Second level information references
  • None

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