抗U1核糖核蛋白抗体在系统性硬化症诊治中的临床意义

目的探讨抗U1核糖核蛋白（U1RNP）抗体在系统性硬化症（SSc）诊治中的价值。方法前瞻性序贯纳入在北京协和医院入选欧洲抗风湿病联盟硬皮病试验研究组（EUSTAR）的131例中国汉族SSc患者，记录其临床表现、脏器损伤和实验室检查结果（包括抗U1RNP抗体的检测结果），分析抗U1RNP抗体阳性组与阴性组临床特征及实验室指标的特点。结果131例SSc患者中含87例弥漫型SSc、36例局限型SSc、8例重叠综合征，抗U1RNP抗体阳性率为28．2％（37／131）。弥漫型SSc和局限型SSc的抗U1RNP抗体阳性率相近，分别为28．7％（25／87）和25．0％（9／36），P=0．673。抗U1RNP抗体阳性者较阴性者易重叠系统性红斑狼疮，肺动脉高压和心脏受累发生率增加，白细胞和血小板计数减低（均P〈0．05）；而在皮肤硬化评分及关节炎、肌炎、肺间质病变发牛率上差异无统计学意义（均P〉0．05）。抗U1RNP抗体阴性的SSc有更高的抗DNA拓扑异构酶Ⅰ抗体检出率。结论抗U1RNP抗体是中国SSc患者的常见自身抗体，与其他自身抗体联合检测有助于SSc的诊断，对预测肺动脉高压、心脏和血液系统受累有重要价值。

Clinical significance of U1-nuclear ribonucleoprotein antibodies in Chinese patients with systemic sclerosis

Objective To investigate clinical relevance of U1-nuclear ribonucleoprotein antibody (anti-U1 RNP) in Chinese patients with systemic sclerosis (SSc). Methods In total, 131 Chinese patients with SSc were prospectively and consecutively recruited into Scleroderma Trials and Research Group sponsored by European League Against Rheumatism (EUSTAR) from clinical database of Peking Union Medical College Hospital ( PUMCH). Their clinical features, visceral lesions and laboratory findings including detection of anti- U1 RNP were recorded. Relevance between existing anti-U1 RNP and clinical characteristics and other laboratory indicators of SSc was analyzed statistically. Results Eighty-seven of the 131 patients presented as diffused SSc (dSSc) , 36 limited SSc ( lSSc) and eight SSc/systemic lupus erythematosus (SLE) overlap syndrome, with positive rate of anti-U, RNP of 28. 2 (37/131) percent in patients with SSc. Positive rate of anti-U1 RNP in dSSc was similar to that in lSSc groups 28. 7% (25/87) and 25. 0% ( 9/36 ) , respectively, P = 0. 673]. But significantly higher prevalence of pulmonary arterial hypertension and cardiac involvement, and less white blood cell and platelets counts were observed in the patients with positive anti-U1 RNP than in those with negative anti-U1 RNP (all P < 0. 05 ). There was no statistically significant difference in skin sclerosis score and prevalence of arthritis, myositis and interstitial lung disease was observed between those with positive and negative anti-U1 RNP ( P > 0. 05 ) . Detection rate of anti-DNA topoisomerase I ( Scl-70 antibody) was higher in SSc patients with negative anti-U, RNP. Conclusions U1 RNP antibody is a common antibody in Chinese patients with SSc, and detection for it combined with other autoantibodies will be helpful for diagnosing of SSc and predicting development of pulmonary arterial hypertension, cardiac and hematological involvements in them.