J Rheumatol:抗合成酶综合征患者的临床特征及预后

2017-05-05 xiangting MedSci原创

这项研究调查了具有不同抗体的抗合成酶综合征患者的临床表型和结果,突出了抗PL7抗体与RP-ILD之间的联系。

这项研究旨在比较中国不同抗合成酶抗体患者的临床特征,并确定长期预后。

研究人员回顾性分析了124例抗合成酶综合征患者,获取医疗记录、实验室检查结果和计算机断层扫描图像。

研究的抗合成酶抗体是抗Jo1(n=62),抗PL7(n=31),抗PL12(n=12)和抗EJ(n=19)。其中间质性肺疾病(ILD)的总发生率达94.4%。11例患者(8.9%)发展为快速进展性ILD(RP-ILD)。8例(6.5%)患有恶性肿瘤。在具有抗PL7的抗合成酶综合征患者中,RP-ILD比不携带抗PL患者7的发病率更高(P=0.028)。抗Ro52阳性的抗合成酶综合征患者的RP-ILD发生频率高于无Ro52患者(P= 0.001)。此外,与抗Ro52共存的抗PL7阳性患者比没有抗Ro52的患者出现更多的RP-ILD(P=0.001)。具有RP-ILD的抗合成酶综合征患者在支气管肺泡灌洗液和血清铁蛋白中的中性粒细胞的比例比没有RP-ILD(P= 0.006,P=0.013)的高。尽管在4个抗合成酶抗体亚组的Kaplan-Meier曲线没有观察到差异(P= 0.349),但在长期随访的早期阶段,抗PL7患者的存活率与其他抗体相比下降得更快。RP-ILD、恶性肿瘤和升高的血清铁蛋白与抗合成酶综合征患者的预后不良相关。

这项研究调查了具有不同抗体的抗合成酶综合征患者的临床表型和结果,突出了抗PL7抗体与RP-ILD之间的联系。

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