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619 例意大利盘状红斑狼疮患者的自身抗体谱及临床特征。

Autoantibody profile and clinical patterns in 619 Italian patients with cutaneous lupus erythematosus.

机构信息

Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy.

Institute of Clinical Physiology, National Research Council, Pisa, Italy.

出版信息

J Eur Acad Dermatol Venereol. 2019 Apr;33(4):742-752. doi: 10.1111/jdv.15147. Epub 2018 Jul 12.

Abstract

BACKGROUND

Anti-nuclear antibodies (ANA), anti-extractable nuclear antigens (ENA) and anti-dsDNA antibodies are often associated with cutaneous lupus erythematosus (CLE), with variable frequency depending on skin subtype. However, specific data based on large case-series on the pathogenetic, diagnostic and prognostic meaning of such autoantibodies are still lacking.

OBJECTIVE

To characterize the correlations between CLE subtypes as well as LE-non-specific skin lesions and their autoantibody pattern.

METHODS

Epidemiological, clinical and immunopathological data of 619 Italian patients with CLE and LE-non-specific skin lesions were analysed. Differences in age, sex, clinical features and autoantibody profile were evaluated in each LE subgroup.

RESULTS

Anti-nuclear antibodies (P < 0.0001), anti-dsDNA (P < 0.0001), ENA (P = 0.001), anti-Sm (P = 0.001), anti-RNP (P = 0.004) and anti-histone (P = 0.005) antibodies were associated with SLE. A strong association between ANA (P < 0.0001) and anti-dsDNA (P < 0.0001) and female gender was also found: positive ANA and positive anti-dsDNA had a higher prevalence among females. Chronic CLE resulted to be negatively associated with ENA (OR = 0.51, P < 0.0001), anti-Ro/SSA (OR = 0.49, P < 0.0001) and anti-dsDNA (OR = 0.37, P < 0.0001). Intermittent CLE resulted to be negatively associated with ENA (OR = 0.50, P = 0.007) and ANA (OR = 0.61, P = 0.025). Subacute CLE resulted to be associated with ENA (OR = 5.19, P < 0.0001), anti-Ro/SSA (OR = 3.83, P < 0.0001), anti-Smith (OR = 2.95, P = 0.004) and anti-RNP (OR = 3.18, P = 0.007). Acute CLE resulted to be strongly associated with anti-dsDNA (OR = 6.0, P < 0.0001) and ANA (OR = 18.1, P < 0.0001). LE-non-specific skin lesions resulted to be significantly associated with systemic involvement. Livedo reticularis was significantly associated with ENA (P = 0.007) and anti-Ro/SSA (P = 0.036). Palpable purpura and periungual telangiectasia were significantly associated with ANA.

CONCLUSION

According to our findings, some well-known associations between CLE subtypes and autoantibody profile were confirmed; moreover, specific association between autoantibodies and LE-non-specific skin lesions was highlighted. A strict association between anti-ENA and anti-Ro/SSA antibodies and livedo reticularis, ANA and palpable purpura, and ANA and periungual telangiectasia was evidenced.

摘要

背景

抗核抗体(ANA)、抗可提取核抗原(ENA)和抗双链 DNA 抗体常与皮肤红斑狼疮(CLE)相关,其频率取决于皮肤亚型。然而,基于大型病例系列的此类自身抗体在发病机制、诊断和预后意义方面的具体数据仍然缺乏。

目的

描述 CLE 亚型以及 LE 非特异性皮肤病变与其自身抗体模式之间的相关性。

方法

分析了 619 例意大利 CLE 和 LE 非特异性皮肤病变患者的流行病学、临床和免疫病理学数据。评估了每个 LE 亚组之间的年龄、性别、临床特征和自身抗体谱的差异。

结果

ANA(P < 0.0001)、抗 dsDNA(P < 0.0001)、ENA(P = 0.001)、抗 Sm(P = 0.001)、抗 RNP(P = 0.004)和抗组蛋白(P = 0.005)抗体与系统性红斑狼疮(SLE)相关。ANA(P < 0.0001)和抗 dsDNA(P < 0.0001)与女性之间也存在强烈关联:ANA 和抗 dsDNA 阳性在女性中更为常见。慢性 CLE 与 ENA(OR = 0.51,P < 0.0001)、抗 Ro/SSA(OR = 0.49,P < 0.0001)和抗 dsDNA(OR = 0.37,P < 0.0001)呈负相关。间歇性 CLE 与 ENA(OR = 0.50,P = 0.007)和 ANA(OR = 0.61,P = 0.025)呈负相关。亚急性 CLE 与 ENA(OR = 5.19,P < 0.0001)、抗 Ro/SSA(OR = 3.83,P < 0.0001)、抗 Smith(OR = 2.95,P = 0.004)和抗 RNP(OR = 3.18,P = 0.007)相关。急性 CLE 与抗 dsDNA(OR = 6.0,P < 0.0001)和 ANA(OR = 18.1,P < 0.0001)呈强相关。LE 非特异性皮肤病变与全身受累显著相关。网状青斑与 ENA(P = 0.007)和抗 Ro/SSA(P = 0.036)显著相关。可触及性紫癜和甲周毛细血管扩张与 ANA 显著相关。

结论

根据我们的发现,证实了 CLE 亚型与自身抗体谱之间的一些已知关联;此外,还强调了自身抗体与 LE 非特异性皮肤病变之间的特定关联。ANA 和抗 Ro/SSA 抗体与网状青斑、ANA 和可触及性紫癜、ANA 和甲周毛细血管扩张之间存在密切关联。

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