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| Previous Nomenclature | Kinetochore |
| Description | Discrete coarse speckles (40-80/cell) scattered in interphase cells and aligned at the chromatin mass on mitotic cells. e.g. anti-CENP-B |
| Antigen Association | CENP-A/B (C) |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ 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) |
| First level information references |
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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 |
| Second level information |
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▶ 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 (10-11) 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. |
| Second level information references |
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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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