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AC-5-粗颗粒型

同义词 剪接体/核基质 spliceosome/nuclear matrix
描述

细胞核呈粗颗粒样荧光，核仁部分阳性。分裂期细胞（中期，后期和末期）浓缩染色体阴性。如：抗-Sm抗体，抗-U1RNP抗体。

 Coarse speckles across all nucleoplasm. The nucleoli may be stained or not stained. Mitotic cells (metaphase, anaphase, and telophase) have the chromatin mass not stained. e.g. anti-Sm, anti-U1 RNP
抗原相关性 hnRNP, U1RNP, Sm, RNA聚合酶III hnRNP, U1RNP, Sm, RNA polymerase III

  • 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


  • ►在不同的系统性自身免疫性风湿疾病中有不同程度的表现，特别是系统性红斑狼疮、系统性硬化症、混合性结缔组织病、系统性硬化症-自身免疫性肌病重叠综合征和未分化结缔组织病（例如，具有风湿症状但未明确诊断为系统性自身免疫性风湿病的患者）(29)

    ►对于临床疑似系统性红斑狼疮的患者，建议后续检测抗Sm和U1RNP抗体；这些抗原通常包含在常规的抗可溶性抗原谱检测中；系统性红斑狼疮的分类标准包含抗Sm抗体(16,30,31)

    ►对于临床疑似系统性硬化症的患者，建议后续检测抗RNApol III抗体（例如，系统性硬化症谱*）；抗RNApol III抗体包含于系统性硬化症的分类标准中(8)

    ►对于临床疑似混合性结缔组织病的患者，建议后续检测抗U1RNP抗体。U1RNP抗原通常包含在常规的ENA谱检测中；抗U1RNP抗体包含于混合性结缔组织病的诊断标准中(32)

    ►对于临床疑似系统性硬化症-自身免疫性肌病重叠综合征的患者，建议后续检测抗U1RNP抗体和抗Ku抗体；U1RNP抗原包含在常规的抗可溶性抗原谱检测中，抗Ku抗体则包含于疾病特异性免疫检测中（例如，炎症性肌病谱*和系统性硬化症谱*）(26,33)

    ►对于一般人群中的非系统性自身免疫性风湿疾病个体，AC-5核型的存在与上述自身抗原无关，且通常抗体滴度低。

    *炎症性肌病谱、系统性硬化症谱和（扩展的）肝病谱的应用可能仅限于专业的临床实验室。



    ► Present to a varying degree in distinct SARD, in particular SLE, SSc, MCTD, SSc-AIM overlap syndrome, and UCTD (i.e, patients with rheumatic symptoms without a definite SARD diagnosis) (29)

    ► If SLE is clinically suspected, it is recommended to perform follow-up tests for anti-Sm and anti-U1RNP antibodies; these antigens are commonly included in the routine ENA profile; anti-Sm antibodies are included in the classification criteria for SLE (16, 30, 31)

    ► If SSc is clinically suspected, it is recommended to perform a follow-up test for anti-RNApol III antibodies (e.g., SSc profile*); the anti-RNApol III antibodies are included in the classification criteria for SSc (8)

    ► If MCTD is clinically suspected, it is recommended to perform a follow-up test for anti-U1RNP antibodies; the antigen is commonly included in the routine ENA profile; anti-U1RNP antibodies are included in the diagnostic criteria for MCTD (32)

    ► If the SSc-AIM overlap syndrome is clinically suspected, it is recommended to perform follow-up tests for anti-U1RNP and anti-Ku antibodies; these antigens are included in the routine ENA profile (U1RNP), or in disease specific immunoassays (Ku, i.e., inflammatory myopathy profile* and SSc profile*) (26, 33)

    ► In non-SARD individuals in the general population, the presence of the AC-5 pattern is not associated with the autoantigens mentioned above and most often concerns low antibody titers

    *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


  • 8. van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of rheumatology/European League against rheumatism collaborative initiative. Ann Rheum Dis 2013;72:1747–55.

    16. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–86.

    26. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med 2016;280:8–23.

    29. Northway JD, Tan EM. Differentiation of antinuclear antibodies giving Speckled staining patterns in immunofluorescence. Clin Immunol Immunopathol 1972;1:140–54.

    30, Satoh M, Fritzler MJ, Chan EKL. Antihistone and antispliceosomal antibodies. In: Lahita RG, Tsokos G, Buyon JP, et al, eds. Systemic lupus erythematosus. San Diego: Academic Press, 2011: 275–92.

    31. Satoh M, Vázquez-Del Mercado M, Chan EK. Clinical interpretation of antinuclear antibody tests in systemic rheumatic diseases. Mod Rheumatol 2009;19:219–28.

    32. Sharp GC, Irvin WS, Tan EM, et al. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med 1972;52:148–59.



    8. van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of rheumatology/European League against rheumatism collaborative initiative. Ann Rheum Dis 2013;72:1747–55.

    16. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–86.

    26. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med 2016;280:8–23.

    29. Northway JD, Tan EM. Differentiation of antinuclear antibodies giving Speckled staining patterns in immunofluorescence. Clin Immunol Immunopathol 1972;1:140–54.

    30, Satoh M, Fritzler MJ, Chan EKL. Antihistone and antispliceosomal antibodies. In: Lahita RG, Tsokos G, Buyon JP, et al, eds. Systemic lupus erythematosus. San Diego: Academic Press, 2011: 275–92.

    31. Satoh M, Vázquez-Del Mercado M, Chan EK. Clinical interpretation of antinuclear antibody tests in systemic rheumatic diseases. Mod Rheumatol 2009;19:219–28.

    32. Sharp GC, Irvin WS, Tan EM, et al. Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med 1972;52:148–59.


  • 二级信息
    Second level information


  • ►少数情况下，AC-5核型相关的自身抗体可能为非U1RNP的RNP，例如U2RNP（与系统性硬化症-自身免疫性肌病重叠综合征相关）或U11/U12RNP（与系统性硬化症相关）；这些自身抗体可以通过免疫沉淀反应进行检测（13,14）

    注：这些自身抗体的特异性免疫检测目前尚未商品化。



    ► Occasionally, autoantibodies revealing the AC‐5 pattern are reactive with RNP other than U1RNP, for instance U2RNP (associated with SSc‐AIM overlap syndrome) or U11/U12RNP (associated with SSc); these autoantibodies can be detected by immunoprecipitation (13, 14)

    Note: Specific immunoassays for these autoantibodies are currently not commercially available.

  • 二级信息参考文献
    Second level information references


  • 13. Mimori T, Hinterberger M, Pettersson I, et al. Autoantibodies to the U2 small nuclear ribonucleoprotein in a patient with scleroderma‐polymyositis overlap syndrome. J Biol Chem 1984;259:560‐5.

    14. Fretig N, Domsic RT, Rodriguez‐Reyna T, et al. Anti‐U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. Arthritis Rheum 2009;61:958‐65.



    13. Mimori T, Hinterberger M, Pettersson I, et al. Autoantibodies to the U2 small nuclear ribonucleoprotein in a patient with scleroderma‐polymyositis overlap syndrome. J Biol Chem 1984;259:560‐5.

    14. Fretig N, Domsic RT, Rodriguez‐Reyna T, et al. Anti‐U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. Arthritis Rheum 2009;61:958‐65.


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