【摘要】 目的描述抗原纤维蛋白抗体阳性的系统性硬化患者临床特点,揭示其与临床特点的相关性。方法采用酶联免疫吸附法检测患者血清中抗原纤维蛋白抗体,使用logistic回归分析抗原纤维蛋白抗体与患者临床和自身抗体的相关性。结果 216名系统性硬化患者中,23例患者抗原纤维蛋白抗体阳性,抗原纤维蛋白抗体阳性组与阴性组之间年龄、病程、性别组成无差异(P>0.05);抗原纤维蛋白抗体与患者的雷诺氏现象、肌炎、间质性肺炎、心脏受累、肾危象无关(P>0.05),而与患者的弥漫型皮肤受累(OR=3.756,95%CI 1.440-9.795,P=0.007)和肺动脉高压(OR=11.945,95%CI 4.538-31.438,P=0.000)相关;抗原纤维蛋白抗体与抗拓扑异构酶抗体(OR=2.562,95%CI 1.013-6.482,P=0.047)和抗U1RNP抗体(OR=4.010,95%CI 1.365-11.780,P=0.012)相关;抗原纤维蛋白抗体与轻度(OR=10.74,95%CI 2.49-46.39,P=0.001)、中度(OR=3.57,95%CI 1.16-10.97,P=0.026)和重度肺动脉高压(OR=3.44,95%CI 1.01-11.71,P=0.048)均显著相关。结论抗原纤维蛋白抗体阳性的系统性硬化患者易发生弥漫型皮肤硬化和肺动脉高压,需严密监测。更多还原

【Abstract】 Objective To explore the clinical characteristics of systemic sclerosis with a positive antifibrillarin antibody and to correlate the antibody status with clinical manifestations.Methods Antifibrillarin antibody were detected by enzyme-linked immunosorbent assay,logistic analysis were employed to correlate the antifibrillarin antibody with clinical manifestations and autoantibody profiles of systemic scelrosis.Results 216 systemic sclerosis patients were recruited,23 of which were positive for antifibrillarin antibody.No difference in age,disease duration and sex were found between patients with and without antifibrillarin antibody(P>0.05);anti-fibrillarin antibody were not correlated with Raynaud’s phenomenon,myositis,interstitial pneumonia,cardiac involvement and kidney crisis(P>0.05),but is associated with diffuse skin involvement(OR=3.756,95%CI 1.440-9.795,P=0.007) and pulmonary artery hypertension(OR=11.945,95%CI 4.538-31.438,P=0.000);anti-fibrillarin antibody were associated with anti-topoisomerase I antibody(OR= 2.562,95%CI 1.013-6.482,P=0.047) and anti-U1RNPantibody(OR=4.010,95% CI 1.365-11.780,P=0.012);anti-fibrillarin antibody was correlated with mild(OR=10.74,95%CI 2.49-46.39,P=0.001),moderate(OR=3.57,95%CI 1.16-10.97,P=0.026) and severe pulmonary artery hypertension(OR=3.44,95%CI 1.01-11.71,P=0.048).Conclusion Systemic sclerosis patients with a positive antifibrillarin antibody is at increased risk to develop diffuse scleroderma and pulmonary artery hypertension,and thus merits close monitoring.更多还原