简体中文

AC-3-着丝点型

同义词 着丝粒 Kinetochore
描述
间期细胞核内可见散在分布的离散型粗颗粒斑点（40–80个/细胞），有丝分裂期细胞中这些斑点沿染色质团内部呈线性排列。

Discrete coarse speckles (40-80/cell) dispersed throughout interphase cells and aligned within the chromatin mass of mitotic cells
抗原相关性 着丝粒蛋白CENP-A、CENP-B及CENP-C Centromere proteins CENP-A, B, C

  • 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

  • ►该核型常见于局限性皮肤型系统性硬化症（lcSSc）患者，已被纳入系统性硬化症（SSc）的分类标准[1–3]。

    ►AC-3核型与雷诺现象联合出现时，可预测lcSSc的发生[1,3]。

    ►该核型与抗CENP-B抗体高度相关；尤其在抗体滴度较低的情况下，建议通过抗原特异性固相免疫分析法进行确认。

    ►此外，AC-3核型亦偶见于部分原发性胆汁性胆管炎（PBC）患者；其中部分患者可能同时呈现SSc与PBC的临床特征。


    ▶Commonly found in patients with limited cutaneous systemic sclerosis (lcSSc), included in the classification criteria for systemic sclerosis (SSc) [1-3]
    ▶In combination with Raynaud’s phenomenon, the AC-3 pattern is predictive of the onset of lcSSc [1, 3]
    ▶Strongly associated with antibodies to CENP-B; especially in case of low titers, confirmation by an antigen-specific solid phase immunoassay is recommended
    ▶The AC-3 pattern is also rarely observed in a subset of patients with primary biliary cholangitis (PBC); some of these patients may present features of SSc in addition to PBC
  • 二级信息
    Second level information

  • ►AC-3核型偶见于原发性干燥综合征（SjD）患者；此类患者常表现为轻度SSc特征，同时伴有典型的SjD临床表现，包括严重的腺体功能障碍以及较高的淋巴瘤发生风险[4–7]。

    ►AC-3核型亦见于部分系统性红斑狼疮患者；这些患者通常存在一定程度的SSc重叠表现[8]。

    ►大多数AC-3核型阳性血清可与CENP-B及CENP-A反应；常规固相免疫分析谱中通常不包含CENP-A，但该抗体已纳入SSc特异性检测谱[9,10]。

    ►部分AC-3核型阳性且抗CENP-B检测阴性的样本，其抗CENP-A抗体可能呈阳性[11,12]。

    ►此外，已有文献报道在SSc及SjD患者中检出抗CENP-C抗体[13,14]。



    ▶The AC-3 pattern is reported in rare patients with primary Sjögren’s disease (SjD); these patients show mild SSc features and a full-blown SjD clinical presentation, with severe exocrine glandular dysfunction, and higher risk for lymphoma [4-7]
    ▶The AC-3 pattern is also reported in a subset of patients with systemic lupus erythematosus; these patients often have some degree of overlap with SSc [8]
    ▶Most sera with the AC-3 pattern react with CENP-B and CENP-A; CENP-A is not included in most routine solid phase immunoassay profiles, but is available in SSc specific profiles [9, 10]
    ▶Some AC-3 samples with negative anti-CENP-B test may be positive for CENP-A antibodies [11, 12]
    ▶Antibodies to CENP-C have been reported in patients with SSc and SjD [13, 14]
  • 参考文献
  • 1.Senécal JL, Koenig M, Archambault G, Hoa S. Criteria for the pathogenicity of anticentromere (anti-CENP-B) autoantibodies in systemic sclerosis: comment on the article by van Leeuwen et al. Arthritis Rheumatol. 2022;74:1606-7
    2.van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, 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
    3.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
    4.Bournia VK, Diamanti KD, Vlachoyiannopoulos PG, Moutsopoulos HM. Anticentromere antibody positive Sjogren's Syndrome: a retrospective descriptive analysis. Arthritis Res Ther. 2010;12:R47
    5.Lee KE, Kang JH, Lee JW, Wen L, Park DJ, Kim TJ, Park YW, Lee SS. Anti-centromere antibody-positive Sjogren's syndrome: A distinct clinical subgroup? Int J Rheum Dis. 2015;18:776-82
    6.Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, Luciano N, Talarico R, et al. Overlap of ACA-positive systemic sclerosis and Sjogren's syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma. Clin Exp Rheumatol. 2013;31:272-80
    7.Baer AN, Medrano L, McAdams-DeMarco M, Gniadek TJ. Association of Anticentromere Antibodies With More Severe Exocrine Glandular Dysfunction in Sjogren's Syndrome: Analysis of the Sjogren's International Collaborative Clinical Alliance Cohort. Arthritis Care Res (Hoboken). 2016;68:1554-9
    8.Nakano M, Ohuchi Y, Hasegawa H, Kuroda T, Ito S, Gejyo F. Clinical significance of anticentromere antibodies in patients with systemic lupus erythematosus. J Rheumatol. 2000;27:1403-7
    9.Perosa F, Prete M, Di Lernia G, Ostuni C, Favoino E, Valentini G. Anti-centromere protein A antibodies in systemic sclerosis: Significance and origin. Autoimmun Rev. 2016;15:102-9
    10.Mahler M, Maes L, Blockmans D, Westhovens R, Bossuyt X, Riemekasten G, Schneider S, Hiepe F, et al. Clinical and serological evaluation of a novel CENP-A peptide based ELISA. Arthritis Res Ther. 2010;12:R99
    11.Russo K, Hoch S, Dima C, Varga J, Teodorescu M. Circulating anticentromere CENP-A and CENP-B antibodies in patients with diffuse and limited systemic sclerosis, systemic lupus erythematosus, and rheumatoid arthritis. J Rheumatol. 2000;27:142-8
    12.Hudson M, Mahler M, Pope J, You D, Tatibouet S, Steele R, Baron M, Investigators of the Canadian Scleroderma Research G, et al. Clinical correlates of CENP-A and CENP-B antibodies in a large cohort of patients with systemic sclerosis. J Rheumatol. 2012;39:787-94
    13.Pillemer SR, Casciola-Rosen L, Baum BJ, Rosen A, Gelber AC. Centromere protein C is a target of autoantibodies in Sjogren's syndrome and is uniformly associated with antibodies to Ro and La. J Rheumatol. 2004;31:1121-5

    14.Gelber AC, Pillemer SR, Baum BJ, Wigley FM, Hummers LK, Morris S, Rosen A, Casciola-Rosen L. Distinct recognition of antibodies to centromere proteins in primary Sjogren's syndrome compared with limited scleroderma. Ann Rheum Dis. 2006;65:1028-32

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