PT - JOURNAL ARTICLE AU - JOSE LUIS CALLEJAS-RUBIO AU - EDUARDO MORENO-ESCOBAR AU - PILAR MARTÍN de la FUENTE AU - LOURDES LÓPEZ PÉREZ AU - RAQUEL RIOS FERNÁNDEZ AU - DANIEL SÁNCHEZ-CANO AU - JOSÉ POMARES MORA AU - NORBERTO ORTEGO-CENTENO TI - Prevalence of Exercise Pulmonary Arterial Hypertension in Scleroderma DP - 2008 Sep 01 TA - The Journal of Rheumatology PG - 1812--1816 VI - 35 IP - 9 4099 - http://www.jrheum.org/content/35/9/1812.short 4100 - http://www.jrheum.org/content/35/9/1812.full SO - J Rheumatol2008 Sep 01; 35 AB - Objective 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. Methods 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. Results 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. Conclusion 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.