Callejas-Rubio Jose Luis, Moreno-Escobar Eduardo, de la Fuente Pilar Martín, Pérez Lourdes López, Fernández Raquel Rios, Sánchez-Cano Daniel, Mora José Pomares, Ortego-Centeno Norberto
Unidad de Enfermedades Autoinmunes Sistémicas, Granada, Spain.
J Rheumatol. 2008 Sep;35(9):1812-6. Epub 2008 Jul 15.
Pulmonary arterial hypertension (PAH) is a complication of scleroderma (systemic sclerosis, SSc); as soon as PAH develops, the patient's prognosis deteriorates rapidly. Early detection of PAH ensures timely treatment. We investigated the prevalence of exercise-induced PAH in a cohort of patients with SSc, and examined the relation between exercise-induced PAH and clinical characteristics and biochemical markers.
Patients with SSc and normal resting systolic pulmonary arterial pressure (sPAP) were studied. Eligible patients were asked to perform cycloergometer exercise until exhaustion, and exercise sPAP was measured. All patients had their pulmonary function tested and underwent echocardiography at rest. Brain natriuretic peptide (BNP) was also determined.
Forty-one patients with SSc were studied. Mean sPAP at rest was 29.7 mm Hg, rising to a mean of 41.4 mm Hg on exercise. Eleven of 41 patients (26.8%) had sPAP post-exercise > 50 mm Hg and 8/41 (19.5%) > 55 mm Hg. A significant correlation was found between exercise sPAP and DLCO (p = 0.008) and between sPAP and BNP levels (p = 0.04). Pre-existing severe Raynaud's phenomenon was more prevalent (50% vs 20%), DLCO levels lower (78.9 vs 92.7 % predicted), and BNP levels higher (72.6 vs 42.1 pmol/ml) in patients with exercise sPAP > 55 mm Hg.
The prevalence of exercise-induced PAH in patients with scleroderma is high. Patients with lower DLCO and higher levels of BNP are at higher risk of developing higher sPAP. Studies with longterm followup are required to evaluate the risk of developing resting PAH in these patients.
肺动脉高压（PAH）是硬皮病（系统性硬化症，SSc）的一种并发症；一旦PAH发生，患者的预后会迅速恶化。早期检测PAH可确保及时治疗。我们调查了一组SSc患者中运动诱发性PAH的患病率，并研究了运动诱发性PAH与临床特征及生化标志物之间的关系。
对静息收缩期肺动脉压（sPAP）正常的SSc患者进行研究。符合条件的患者被要求进行症状限制性运动平板试验，直至力竭，并测量运动时的sPAP。所有患者均进行了肺功能测试并在静息状态下接受了超声心动图检查。同时还测定了脑钠肽（BNP）。
共研究了41例SSc患者。静息时平均sPAP为29.7mmHg，运动时升至平均41.4mmHg。41例患者中有11例（26.8%）运动后sPAP>50mmHg，8/41（19.5%）>55mmHg。运动sPAP与肺一氧化碳弥散量（DLCO）之间存在显著相关性（p = 0.008），sPAP与BNP水平之间也存在显著相关性（p = 0.04）。运动sPAP>55mmHg的患者中，既往严重雷诺现象更为普遍（50%对20%），DLCO水平更低（预测值的78.9%对92.7%），BNP水平更高（72.6对42.1pmol/ml）。
硬皮病患者中运动诱发性PAH的患病率较高。DLCO较低和BNP水平较高的患者发生较高sPAP的风险更高。需要进行长期随访研究以评估这些患者发生静息性PAH的风险。
