Prevalence of Exercise Pulmonary Arterial Hypertension in Scleroderma

• Published in: Journal of rheumatology 2008-09, Vol.35 (9), p.1812-1816
• Main Authors: CALLEJAS-RUBIO, Jose Luis, MORENO-ESCOBAR, Eduardo, DE LA FUENTE, Pilar Martin, LOPEZ PEREZ, Lourdes, RIOS FERNANDEZ, Raquel, SANCHEZ-CANO, Daniel, POMARES MORA, José, ORTEGO-CENTENO, Norberto
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Blood Pressure Determination; Cross-Sectional Studies; Disease Progression; Diseases of the osteoarticular system; Echocardiography, Doppler; Exercise; Exercise Test; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary - blood; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Male; Medical sciences; Middle Aged; Natriuretic Peptide, Brain - blood; Pneumology; Prevalence; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Respiratory Function Tests; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Systemic - blood; Scleroderma, Systemic - complications; Scleroderma, Systemic - physiopathology; Spain
• ISSN: 0315-162X; 1499-2752

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. Key Indexing Terms: PULMONARY ARTERIAL HYPERTENSION PROGNOSIS SYSTEMIC SCLEROSIS PULMONARY ARTERIAL SYSTOLIC PRESSURE EARLY DETECTION