Royal Free Hospital and University College London, London, UK, and Université de Lille, Centre National de Référence de la Sclérodermie Systémique, Hôpital Claude Huriez, EA2686, INSERM U995, Lille Inflammation Research International Centre, and Fédération Hospitalo-Universitaire Immune-Mediated Inflammatory Diseases and Targeted Therapies (IMMINeNT), Lille, France.
Université de Lille, Fédération Hospitalo-Universitaire IMMINeNT, and Centre Hospitalier Régional Universitaire de Lille, Lille, France.
Arthritis Rheumatol. 2016 Feb;68(2):484-93. doi: 10.1002/art.39432.
Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti-U1 RNP-positive patients with CTD-associated PAH, with a focus on systemic sclerosis (SSc)-associated PAH.
We implemented a prospective database that included patients with CTD-associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti-U1 RNP antibody status. We then assessed whether anti-U1 RNP antibodies could be a prognostic factor in CTD-associated PAH with a focus on SSc-associated PAH.
We studied a total of 342 patients with CTD-associated PAH, of whom 36 (11%) were anti-U1 RNP antibody positive. Anti-U1 RNP-positive patients were younger and less functionally impaired than were anti-U1 RNP-negative patients in CTD- and SSc-associated PAH. Hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients. In CTD-associated PAH, anti-U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18-0.65], P < 0.001). In multivariable analysis, anti-U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20-0.97], P = 0.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc-associated PAH, although the association between anti-U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22-1.02], P = 0.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20-1.11], P = 0.085) analyses.
Anti-U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD- and SSc-associated PAH. While hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients, our results suggest that anti-U1 RNP positivity could be a factor protecting against mortality in CTD- and SSc-associated PAH.
肺动脉高压（PAH）是结缔组织疾病（CTD）的严重并发症。本研究旨在探讨抗 U1 RNP 阳性的 CTD 相关 PAH 患者的临床和血流动力学特征及生存率，重点关注系统性硬化症（SSc）相关 PAH。
我们建立了一个前瞻性数据库，纳入了有临床、自身抗体和死亡率数据的 CTD 相关 PAH 患者。我们比较了临床和血流动力学特征与抗 U1 RNP 抗体状态的关系。然后评估了抗 U1 RNP 抗体是否可以作为 CTD 相关 PAH 的预后因素，重点关注 SSc 相关 PAH。
我们共研究了 342 例 CTD 相关 PAH 患者，其中 36 例（11%）为抗 U1 RNP 抗体阳性。与 CTD 相关 PAH 患者相比，抗 U1 RNP 阳性患者在 CTD 和 SSc 相关 PAH 中年龄更小，功能障碍程度更低。抗 U1 RNP 阳性和抗 U1 RNP 阴性患者的血流动力学参数相似。在 CTD 相关 PAH 中，单变量分析显示抗 U1 RNP 阳性与死亡率降低相关（风险比 0.34[95%置信区间 0.18-0.65]，P<0.001）。多变量分析显示，抗 U1 RNP 阳性也与死亡率降低相关（风险比 0.44[95%置信区间 0.20-0.97]，P=0.043），独立于年龄、性别、功能参数、肺受累和血流动力学参数。SSc 相关 PAH 中也有类似结果，尽管抗 U1 RNP 阳性与生存之间的关联在单变量（风险比 0.47[95%置信区间 0.22-1.02]，P=0.055）和多变量（风险比 0.47[95%置信区间 0.20-1.11]，P=0.085）分析中均未达到显著水平。
抗 U1 RNP 阳性与 CTD 和 SSc 相关 PAH 的临床特征和生存相关。虽然抗 U1 RNP 阳性和抗 U1 RNP 阴性患者的血流动力学参数相似，但我们的结果表明，抗 U1 RNP 阳性可能是 CTD 和 SSc 相关 PAH 中降低死亡率的一个因素。
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