Semalulu T, Rudski L, Huynh T, Langleben D, Wang M, Fritzler M J, Pope J, Baron M, Hudson M
Department of Medicine, McMaster University, Canada.
Department of Medicine, McGill University, Montreal, Canada; Division of Cardiology, Jewish General Hospital, Montreal, Canada.
Semin Arthritis Rheum. 2020 Dec;50(6):1421-1427. doi: 10.1016/j.semarthrit.2020.02.013. Epub 2020 Mar 2.
Clinical practice guidelines recommend screening all systemic sclerosis (SSc) patients for pulmonary arterial hypertension (PAH) with yearly echocardiograms. There is a paucity of evidence to support these guidelines.
Can a prediction model identify SSc patients with a very low probability of PAH and therefore not requiring annual screening echocardiogram?
We performed a case-control study of 925 unselected SSc subjects nested in a multi-centered, longitudinal cohort. The probability of PAH for each subject was calculated using the results of multivariate logistic regression models. A cut-off was identified for the estimated probability of PAH below which no subject developed PAH (100% sensitivity).
Study subjects were predominantly female (87.5%), with mean (SD) age 58.6 (11.7) years and disease duration of 18.2 (12.2) years. Thirty-seven subjects developed PAH during 5407.97 person-years of observation (incidence rate 0.68 per 100 person-years). Shortness of breath (SOB), diffusing capacity for carbon monoxide (DLCO) and NT-proBNP were independent predictors of PAH. All SSc-PAH cases had a probability of PAH of >1.1%. Subjects below this cut-off, none of whom had PAH, accounted for 46.2% of the study population.
A simple prediction model identified subjects at very low probability of PAH who could potentially forego annual screening echocardiogram. This represents almost half of SSc subjects in a general SSc population. This study, which is the first evidence-based study for the rational use of follow-up echocardiograms in an unselected SSc cohort, requires validation. The scoring system is freely available online at http://pahtool.ladydavis.ca.
临床实践指南建议对所有系统性硬化症（SSc）患者每年进行超声心动图检查以筛查肺动脉高压（PAH）。但支持这些指南的证据不足。
能否通过一个预测模型识别出PAH发生概率极低、因此无需每年进行筛查性超声心动图检查的SSc患者？
我们在一个多中心纵向队列中对925名未经选择的SSc受试者进行了病例对照研究。使用多变量逻辑回归模型的结果计算每个受试者发生PAH的概率。确定了PAH估计概率的一个临界值，低于该临界值没有受试者发生PAH（灵敏度为100%）。
研究对象主要为女性（87.5%），平均（标准差）年龄58.6（11.7）岁，病程18.2（12.2）年。在5407.97人年的观察期内，有37名受试者发生了PAH（发病率为每100人年0.68例）。呼吸急促（SOB）、一氧化碳弥散量（DLCO）和NT-前脑钠肽是PAH的独立预测因素。所有SSc-PAH病例发生PAH的概率均>1.1%。低于该临界值的受试者中没有一人发生PAH，占研究人群的46.2%。
一个简单的预测模型识别出了PAH发生概率极低、可能无需每年进行筛查性超声心动图检查的受试者。这几乎占普通SSc人群中SSc受试者的一半。这项研究是关于在未经选择的SSc队列中合理使用随访超声心动图的第一项基于证据的研究，需要进行验证。评分系统可在http://pahtool.ladydavis.ca免费在线获取。
