【摘要】 目的 研究抗RNA多聚酶Ⅲ抗体(anti-RNA polymeraseⅢantibodies, ARA)相关系统性硬化(systemic sclerosis, SSc)患者的临床特点。方法 纳入2017年1月至2020年12月确诊的SSc患者56例，采用线性免疫印迹法检测ARA,分析其临床特点。结果 56例SSc患者中，ARA(+)8例(14.3%),男∶女为5∶3,中位年龄54岁，中位病程12个月，临床分型以弥漫性SSc为主(62.5%)。其中雷诺现象(62.5%)、甲周毛细血管特征性改变(62.5%)、肺间质纤维化(50.0%)是ARA(+)患者最常见的三大临床表现。与ARA(-)组相比，ARA(+)组男性比例显著升高(62.5%vs.10.4%,P=0.003),病程更短(12月vs.66月，P=0.006),并发肿瘤的概率更高(37.5%vs.0%,P=0.002),而雷诺现象发生率更低(62.5%vs.97.9%,P=0.008)。将患者按ARA(+)、ATA(+)、ACA(+)分为三组，并行两两组间比较发现：ARA(+)组与ATA(+)组相比，各项临床特征差异无统计学意义(P>0.017);与ACA(+)组相比，ARA(+)组男性多见，肿瘤更常见，雷诺现象较少见，差异均具有统计学意义(P<0.017)。结论 ARA(+)SSc在临床上更需早期识别并积极诊治，ARA应作为SSc临床常规检测手段。更多还原

【Abstract】 Objective To observe the clinical features of systemic sclerosis(SSc) patients with anti-RNA polymerase Ⅲ antibodies(ARA).Methods Immunoblotting assay was used to detect the presence of ARA in 56 patients with SSc, who were enrolled from January 2017 to December 2020.The clinical manifestations and characteristics of ARA positive patients were analyzed.Results Among 56 patients with SSc, 8 cases(14.3%) were positive for ARA and male: female ratio was 5∶3.Median age was 54 years and median duration was 12 months.The main clinical classification was diffuse cutaneous SSc(62.5%).Raynaud’s phenomenon(62.5%),characteristic changes of nailfold videocapillaroscopy(62.5%),and pulmonary interstitial fibrosis(50.0%) were the most common clinical manifestations of ARA positive patients.The patients with ARA had significantly higher proportion of men gender(62.5% vs. 10.4%,P=0.003),higher probability of malignancy(37.5% vs. 0%,P=0.002),but shorter course of the disease(12 months vs. 66 months, P=0.006) and lower prevalence of Raynaud’s phenomenon(62.5% vs. 97.9%,P=0.008).If the patients were divided into ARA positive group, ATA positive group, and ACA positive group, and then pairwise comparisons were performed.There was no statistically significance on above items between ARA positive group and ATA positive group(P>0.017).Compared with ACA-positive patients, patients with ARA had less common Raynaud’s phenomenon, more higher proportion of male, more higher probability of malignancy(P<0.017).Conclusions Early recognition and timely diagnosis are necessary for SSc patients with ARA.ARA should be considered as a routine test for high-risk SSc patients.更多还原