AC-3: Centromere型（セントロメア型）

x400

x1,000
間期

間期核は40～80個の散在した荒い斑点状に染色される

核小体

陰性

分裂期

分裂期クロマチンも散在した荒い斑点状に染色される

疑 われる疾患●

限局皮膚硬化型SSc

旧名：Discrete speckled型（散在斑紋型）

MBL 関連製品●PBC

CLEIA法ステイシア MEBLux™テスト CENP-B

推 奨フォローアップ検査●

抗CENP-B抗体

関 連抗原
CENP-A/B (C)メモ
AC-3 型は抗 CENP-B抗体と強く関連する。HEp-2 IIF検査が低力価の場合、抗 CENP-B抗体の固相免疫測定法が推奨される[3]。
AC-3 型を呈するPBC患者は多くの場合、SScを併発する[3]。
AC-3 型は SS患者のサブセットで報告される。これらの患者は軽い SS の特徴を示すが、より重度の外分泌腺機能不全やリンパ腫の高リスクなどの末期SSの臨床症状を有することもある[7, 11,14,15]。
AC-3型はSLE患者のサブセットでも報告される。これらの患者はしばしばある程度のSScとのオーバーラップを有する[1]。
AC-3型を呈する大抵の血清はCENP-AおよびCENP-Bに反応する[8, 16]。
AC-3型陽性の稀なケースでは、SSc患者のCENP-B陰性血清が抗CENP-A抗体強陽性となる場合がある[2, 9]。
CENP- Cに対する自己抗体はSScおよびSSの患者で報告される[4, 5]。
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ANA-IIF Pattern Library

参考文献
1. Nakano M, Ohuchi Y, Hasegawa H, Kuroda T, Ito S, Gejyo F. Clinicalsigniﬁcance of anticentromere antibodies in patients with systemic
lupus erythematosus. J Rheumatol. 2000;27(6):1403-7.
2. Russo K, Hoch S, Dima C, Varga J, Teodorescu M. Circulating
anticentromere CENP-A and CENP-B antibodies in patients withdiffuse and limited systemic sclerosis, systemic lupus erythematosus,
and rheumatoid arthritis. J Rheumatol. 2000;27(1):142-8.
3. Conrad K, Schößler W, Hiepe F, Fritzler MJ. Autoantibodies insystemic autoimmune diseases: a diagnostic reference: Pabst
Science Publishers; 2002.
4. 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(6):1121-5.
5. Gelber AC, Pillemer SR, Baum BJ, Wigley FM, Hummers LK, Morris S,
et al. Distinct recognition of antibodies to centromere proteins inprimary Sjogren's syndrome compared with limited scleroderma.
Ann Rheum Dis. 2006;65(8):1028-32.
6. Invernizzi P, Lleo A, Podda M. Interpreting serological tests indiagnosing autoimmune liver diseases. Semin Liver Dis. 2007;27(2):161-72.

7. Bournia VK, Diamanti KD, Vlachoyiannopoulos PG, Moutsopoulos
HM. Anticentromere antibody positive Sjogren's Syndrome: aretrospective descriptive analysis. Arthritis Res Ther. 2010;12(2):R47.
8. Mahler M, Maes L, Blockmans D, Westhovens R, Bossuyt X,Riemekasten G, et al. Clinical and serological evaluation of a novel
CENP-A peptide based ELISA. Arthritis Res Ther. 2010;12(3):R99.
9. Hudson M, Mahler M, Pope J, You D, Tatibouet S, Steele R, et al.
Clinical correlates of CENP-A and CENP-B antibodies in a large cohortof patients with systemic sclerosis. J Rheumatol. 2012;39(4):787-94.
10. Johnson SR, Fransen J, Khanna D, Baron M, van den Hoogen F,Medsger TA, Jr., et al. Validation of potential classiﬁcation criteria forsystemic sclerosis. Arthritis Care Res (Hoboken). 2012;64(3):358-67.
11. Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F,
et al. Overlap of ACA-positive systemic sclerosis and Sjogren's
syndrome: a distinct clinical entity with mild organ involvement butat high risk of lymphoma. Clin Exp Rheumatol. 2013;31(2):272-80.
12. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M,Tyndall A, et al. 2013 classiﬁcation criteria for systemic sclerosis: anAmerican college of rheumatology/European league against
rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55.
13. Chan EK, Damoiseaux J, Carballo OG, Conrad K, de Melo Cruvinel W,Francescantonio PL, et al. Report of the First International
Consensus on Standardized Nomenclature of Antinuclear Antibody
HEp-2 Cell Patterns 2014-2015. Front Immunol. 2015;6:412.
14. Lee KE, Kang JH, Lee JW, Wen L, Park DJ, Kim TJ, et al.
Anti-centromere antibody-positive Sjogren's syndrome: A distinct
clinical subgroup? Int J Rheum Dis. 2015;18(7):776-82.
15. Baer AN, Medrano L, McAdams-DeMarco M, Gniadek TJ. Associationof 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(10):1554-9.
16. 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(1):102-9.
17. Damoiseaux J, Andrade LEC, Carballo OG, Conrad K, Francescantonio
PLC, Fritzler MJ, et al. Clinical relevance of HEp-2 indirect
immunoﬂuorescent patterns: the International Consensus on ANA
patterns (ICAP) perspective. Ann Rheum Dis. 2019;78(7):879-89.

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