系统性硬化症中特定自身抗体的血清水平预示着更严重的皮肤受累。
The serum levels of specific autoantibodies in systemic sclerosis predict a more severe skin involvement.
作者信息
Dengler Hannah, Vonow-Eisenring Maya, Becker Mike Oliver, Dobrota Rucsandra, Mihai Carina, Muraru Sinziana, Hoffmann-Vold Anna-Maria, Distler Oliver, Bruni Cosimo, Elhai Muriel
机构信息
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Department of Immunology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
出版信息
J Scleroderma Relat Disord. 2025 Jul 28:23971983251357991. doi: 10.1177/23971983251357991.
BACKGROUND
Systemic sclerosis is a severe autoimmune disease characterized by fibrosis of the skin and internal organs. Systemic sclerosis is associated with the presence of three specific autoantibodies: anti-topoisomerase I, anti-centromere, and anti-RNA polymerase III autoantibodies, which have also been identified as prognostic factors. However, it remains unknown whether the prognosis also varies based on their serum levels.
OBJECTIVES
We aimed to assess the value of serum levels of systemic sclerosis-specific autoantibodies as biomarkers of disease severity and progression in systemic sclerosis.
DESIGN
We conducted a post hoc longitudinal analysis of data of systemic sclerosis patients included in the Zurich EUSTAR cohort, who were positive for at least one of the three systemic sclerosis-specific autoantibodies.
METHODS
The association between the levels of systemic sclerosis-specific autoantibodies and disease severity at baseline and during the follow-up was assessed by univariable and multivariable logistic and linear regressions.
RESULTS
The serum levels of anti-topoisomerase I autoantibodies [β = 0.032 (95% confidence interval = 0.014 to 0.049), p < 0.001], anti-centromere [β = 0.002 (95% confidence interval = 0.001 to 0.003), p < 0.001], and anti-RNA polymerase III autoantibodies [β = 0.143 (95% confidence interval = 0.066 to 0.220), p < 0.001] were associated with the modified Rodnan Skin Score in univariable analysis at baseline. For anti-centromere [β = 0.002 (95% confidence interval = 0.001 to 0.003), p < 0.001] and anti-RNA polymerase III autoantibodies [β = 0.135 (95% confidence interval = 0.053 to 0.217), p = 0.002], this association also remained significant in multivariable analysis. In the longitudinal analysis, the levels of the three systemic sclerosis-specific autoantibodies did not predict changes in mRSS over 1 year.
CONCLUSION
Increased serum levels of all three autoantibodies predicted a more severe skin fibrosis. The results underscore the relevance of measuring the levels of systemic sclerosis-specific autoantibodies to enhance risk stratification in systemic sclerosis, with particular focus on skin involvement.
背景
系统性硬化症是一种严重的自身免疫性疾病，其特征为皮肤和内脏器官纤维化。系统性硬化症与三种特定自身抗体的存在相关：抗拓扑异构酶I、抗着丝粒和抗RNA聚合酶III自身抗体，这些抗体也被确定为预后因素。然而，基于它们的血清水平预后是否也存在差异仍不清楚。
目的
我们旨在评估系统性硬化症特异性自身抗体的血清水平作为系统性硬化症疾病严重程度和进展的生物标志物的价值。
设计
我们对苏黎世EUSTAR队列中至少对三种系统性硬化症特异性自身抗体中的一种呈阳性的系统性硬化症患者的数据进行了事后纵向分析。
方法
通过单变量和多变量逻辑回归及线性回归评估系统性硬化症特异性自身抗体水平与基线及随访期间疾病严重程度之间的关联。
结果
在基线时的单变量分析中，抗拓扑异构酶I自身抗体的血清水平[β = 0.032（95%置信区间 = 0.014至0.049），p < 0.001]、抗着丝粒[β = 0.002（95%置信区间 = 0.001至0.003），p < 0.001]和抗RNA聚合酶III自身抗体[β = 0.143（95%置信区间 = 0.066至0.220），p < 0.001]与改良Rodnan皮肤评分相关。对于抗着丝粒[β = 0.002（95%置信区间 = 0.001至0.003），p < 0.001]和抗RNA聚合酶III自身抗体[β = 0.135（95%置信区间 = 0.053至0.217），p = 0.002]，这种关联在多变量分析中也仍然显著。在纵向分析中，三种系统性硬化症特异性自身抗体的水平并未预测1年内改良Rodnan皮肤评分的变化。
结论
所有三种自身抗体血清水平升高预示着更严重的皮肤纤维化。结果强调了测量系统性硬化症特异性自身抗体水平对于加强系统性硬化症风险分层的相关性，尤其关注皮肤受累情况。
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