ICAP
AC-3 - 着丝点型
  简体中文 English
同义词 着丝粒 Kinetochore
描述

间期细胞呈现离散型的粗荧光颗粒(40-80/细胞),分裂期细胞的中间位置出现带状的浓缩点状荧光。

Discrete coarse speckles (40-80/cell) scattered in interphase cells and aligned at the chromatin mass on mitotic cells. e.g. anti-CENP-B

抗原相关性 CENP-A/B(C) 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

►常见于局限皮肤型系统性硬化症的患者,因此包含在系统性硬化症的分类标准中(8,15,23)

►结合雷诺现象,AC-3核型可作为预测局限皮肤型系统性硬化症的指标(15,23)

►与抗CENP-B抗体高度相关; CENP-B抗原包含在许多常规的多分析物固相免疫检测谱中(15);尤其是在滴度较低的情况下,建议通过抗原特异性固相免疫检测法进行确认;

►在一部分原发性胆汁性胆管炎患者中也观察到AC-3核型;这些患者通常同时患有系统性硬化症和原发性胆汁性胆管炎(15)

► Commonly found in patients with limited cutaneous SSc, and as such included in the classification criteria for SSc (8, 15, 23)

► In combination with Raynaud phenomenon, the AC-3 pattern is predictive for onset of limited cutaneous SSc (15, 23)

► Strongly associated with antibodies to CENP-B; the CENP-B antigen is included in many routine multi-analyte solid phase immunoassay profiles (15); especially in case of low titers, confirmation by an antigen-specific solid phase immunoassay is recommended;

► The AC-3 pattern is also observed in a subset of patients with PBC; these patients often have both SSc as well as PBC (15)


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.

15. Conrad K, Schössler W, Hiepe F. Autoantibodies in systemic autoimmune diseases: a diagnostic reference. 2. 3th edn. Autoantigens autoantibodies autoimmunity, 2015.

23. Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358–67


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.

15. Conrad K, Schössler W, Hiepe F. Autoantibodies in systemic autoimmune diseases: a diagnostic reference. 2. 3th edn. Autoantigens autoantibodies autoimmunity, 2015.

23. Johnson SR, Fransen J, Khanna D, et al. Validation of potential classification criteria for systemic sclerosis. Arthritis Care Res 2012;64:358–67

二级信息 Second level information

►在部分干燥综合征患者中报告了AC-3核型;这些患者表现出轻度系统性硬化症的特征,但是具有完全的干燥综合征的临床特征、较严重的外分泌腺功能障碍及高危淋巴瘤(1-4)

►在部分系统性红斑狼疮患者中也报告了AC-3核型;这些患者通常与系统性硬化症有一定程度的重叠(5)

►大多数AC-3核型血清与CENP-A和CENP-B反应;在某些疾病特异性固相免疫检测(例如,系统性硬化症谱)中可以检测到针对CENP-A的抗体(6、7)

►在极少数情况下,系统性硬化症患者的AC-3阳性,但CENP-B阴性的血清可能存在抗CENP-A抗体的强阳性(8,9)

►在系统性硬化症和干燥综合征患者中已报道了针对CENP-C的抗体(10-12)

注:CENP-A检测的应用(例如,酶联免疫吸附实验或系统性硬化症谱)可能仅限于专业临床实验室; CENP-C抗体的特异性免疫检测目前尚无商品化试剂。


► The AC-3 pattern is reported in a subset of patients with SjS; these patients show mild SSc features, but a full-blown SjS clinical feature, more severe exocrine glandular dysfunction, and high risk of lymphoma (1-4)
► The AC-3 pattern is also reported in a subset of patients with SLE; these patients often have some degree of overlap with SSc (5)
► Most sera with the AC-3 pattern react with CENP-A and CENP-B; antibodies to CENP-A can be detected in some disease specific solid phase immunoassays (i.e., SSc profile) (6, 7)
► In rare cases AC-3 positive, but CENP-B negative sera of SSc patients may be strongly positive for anti-CENP-A antibodies (8, 9)
► Antibodies to CENP-C have been reported in patients with SSc and SjS (10-12)
Note: Availability of assays for CENP-A, (i.e., ELISA or SSc profile), may be limited to specialty clinical laboratories; specific immunoassays for anti-CENP-C antibodies are currently not commercially available.

二级信息参考文献 Second level information references

1. Vasiliki-Kallipi KB, Diamanti KD, Vlachoyiannopoulos PG, et al. Anticentromere antibody positive Sjögren’s Syndrome: a retrospective descriptive analysis. Arthritis Res Ther 2010;12:R47.

2. Lee KE, Kang JH, Lee JW, et al. Anti-centromere antibody-positive Sjögren’s syndrome: a distinct clinical subgroup? Int J Rheum Dis 2015;18:776-82.

3. Baldini C, Mosca M, Della Rossa A, et al. Overlap of ACA-positive systemic sclerosis and Sjögren’s syndrome: a distinct clinical entity with mild organ involvement but a high risk of lymphoma. Clin Exp Rheumatol 2013;31:272-80.

4. Baer AN, Medrano L, McAdams-Demarco M, et al. Association of anticentromere antibodies with more severe exocrine glandular dysfunction in Sjögren’s syndrome: analysis of the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res 2016;68:1554-9.

5. Nakano M, Ohuchi Y, Hasegawa H, et al. Clinical significance of anticentromere antibodies in patients with systemic lupus erythematosus. J Rheumatol 2000;27:1403-7.

6. Perosa F, Prete M, Di Lernia G, et al. Anti-centromere protein A antibodies in systemic sclerosis: significance and origin. Autoimmun Rev 2016;15:102-9.

7. Mahler M, Maes L, Blockmans D, et al. Clinical and serological evaluation of a novel CENP-A peptide based ELISA. Arthritis Res Ther 2010;12:R99.

8. Russo K, Hoch S, Varga J, et al. Circulating anticentromere CENP-A and CENP-B antibodies in patients with diffuese and limited systemic sclerosis, systemic lupus erythematosis, and rheumatoid arthritis. J Rheumatol 2000;27:142-8.

9. Hudson M, Mahler M, Pope J, 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.

10. Pillemer SR, Casciola-Rosen L, Baum BJ, et al. 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.

11. Gelber AC, Pillemer SR, Baum BJ, et al. Distinct recognition of antibodies to centromere proteins in primary Sjogren's syndrome compared with limited scleroderma. Ann Rheum Dis 2006;65:1028-32.


1. Vasiliki-Kallipi KB, Diamanti KD, Vlachoyiannopoulos PG, et al. Anticentromere antibody positive Sjögren’s Syndrome: a retrospective descriptive analysis. Arthritis Res Ther 2010;12:R47.

2. Lee KE, Kang JH, Lee JW, et al. Anti-centromere antibody-positive Sjögren’s syndrome: a distinct clinical subgroup? Int J Rheum Dis 2015;18:776-82.

3. Baldini C, Mosca M, Della Rossa A, et al. Overlap of ACA-positive systemic sclerosis and Sjögren’s syndrome: a distinct clinical entity with mild organ involvement but a high risk of lymphoma. Clin Exp Rheumatol 2013;31:272-80.

4. Baer AN, Medrano L, McAdams-Demarco M, et al. Association of anticentromere antibodies with more severe exocrine glandular dysfunction in Sjögren’s syndrome: analysis of the Sjögren’s International Collaborative Clinical Alliance Cohort. Arthritis Care Res 2016;68:1554-9.

5. Nakano M, Ohuchi Y, Hasegawa H, et al. Clinical significance of anticentromere antibodies in patients with systemic lupus erythematosus. J Rheumatol 2000;27:1403-7.

6. Perosa F, Prete M, Di Lernia G, et al. Anti-centromere protein A antibodies in systemic sclerosis: significance and origin. Autoimmun Rev 2016;15:102-9.

7. Mahler M, Maes L, Blockmans D, et al. Clinical and serological evaluation of a novel CENP-A peptide based ELISA. Arthritis Res Ther 2010;12:R99.

8. Russo K, Hoch S, Varga J, et al. Circulating anticentromere CENP-A and CENP-B antibodies in patients with diffuese and limited systemic sclerosis, systemic lupus erythematosis, and rheumatoid arthritis. J Rheumatol 2000;27:142-8.

9. Hudson M, Mahler M, Pope J, 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.

10. Pillemer SR, Casciola-Rosen L, Baum BJ, et al. 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.

11. Gelber AC, Pillemer SR, Baum BJ, et al. 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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Online since 19 May 2015