►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)

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

►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.

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.