AC-5: Nuclear large/coarse speckled型（Speckled/斑紋型）

x400

x1,000
間期

核小体

間期核全体に粗い斑点（coarse speckles）が見られる
核小体の染色は一定ではなく、陽性または陰性である

分裂期 分裂期クロマチンは陰性

疑 われる疾患● SSc
SLEMCTD● SSc-筋炎オーバーラップ症候群● UCTD
●●推 奨フォローアップ検査
SLE疑い例● 抗Sm抗体●抗U1RNP抗体

SSc疑い例
抗RNAポリメラーゼ III抗体

メモ
一般集団の非膠原病集団内で、AC-5型の存在は推奨フォ
ローアップ検査に示した自己抗原とは関連せず、通常は低力価を示す。
AC-5型を呈する抗体は時折、U1RNP以外のRNP、例えばU2RNP（SSc-筋炎オーバーラップ症候群と関連）またはU11/U12RNP（SScと関連）と反応する。これらの自己抗体は免疫沈降法で検出できる[4, 6]。

MBL 関連製品
ELISA法MESACUP™ anti-RNA ポリメラーゼ III テスト

CLEIA法ステイシア MEBLux™テスト Sm
ステイシア MEBLux™テスト RNP●MCTD疑い例● 抗U1RNP抗体
SSc-筋炎オーバーラップ症候群疑い例● 抗U1RNP抗体● 抗Ku抗体

関 連抗原
hnRNP、U1RNP、Sm、RNAポリメラーゼ III

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ANA-IIF Pattern Library参 考 文献
1. Northway J, Tan E. Differentiation of antinuclear antibodies giving
speckled staining patterns in immunoﬂuorescence. Clinical
Immunology and Immunopathology. 1972;1(1):140-54.
2. Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. Mixed
connective tissue disease--an apparently distinct rheumatic disease
syndrome associated with a speciﬁc antibody to an extractable
nuclear antigen (ENA). Am J Med. 1972;52(2):148-59.
3. Fritzler MJ, Ali R, Tan EM. Antibodies from patients with mixed
connective tissue disease react with heterogeneous nuclear
ribonucleoprotein or ribonucleic acid (hnRNP/RNA) of the nuclear
matrix. J Immunol. 1984;132(3):1216-22.
4. Mimori T, Hinterberger M, Pettersson I, Steitz JA. Autoantibodies tothe U2 small nuclear ribonucleoprotein in a patient withscleroderma-polymyositis overlap syndrome. J Biol Chem. 1984;259(1):560-5.
5. Steiner G, Skriner K, Smolen JS. Autoantibodies to the A/B proteinsof the heterogeneous nuclear ribonucleoprotein complex: novel tools
for the diagnosis of rheumatic diseases. Int Arch Allergy Immunol.
1996;111(4):314-9.
6. Fertig N, Domsic RT, Rodriguez-Reyna T, Kuwana M, Lucas M,
Medsger TA, Jr., et al. Anti-U11/U12 RNP antibodies in systemicsclerosis: a new serologic marker associated with pulmonary ﬁbrosis.
Arthritis Rheum. 2009;61(7):958-65.
7. Satoh M, Vazquez-Del Mercado M, Chan EK. Clinical interpretation ofantinuclear antibody tests in systemic rheumatic diseases. ModRheumatol. 2009;19(3):219-28.
8. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, et al.
Derivation and validation of the Systemic Lupus International
Collaborating Clinics classiﬁcation criteria for systemic lupus
erythematosus. Arthritis Rheum. 2012;64(8):2677-86.
9. 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 againstrheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55.
10. 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.
11. Betteridge Z, McHugh N. Myositis-speciﬁc autoantibodies: animportant tool to support diagnosis of myositis. J Intern Med.
2016;280(1):8-23.
12. Satoh M, Tanaka S, Ceribelli A, Calise SJ, Chan EK. A Comprehensive
Overview on Myositis-Speciﬁc Antibodies: New and Old Biomarkers
in Idiopathic Inﬂammatory Myopathy. Clin Rev Allergy Immunol.
2017;52(1):1-19.
13. 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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