Abstract

BACKGROUND: Systemic sclerosis (SSc) may be complicated by pulmonary hypertension (PHT), which can occur both in the setting of fibrosing alveolitis or as lone pulmonary vascular disease. Nitric oxide (NO) is a powerful vasodilator and is produced by various cells in the respiratory tract including pulmonary vascular endothelial cells and can be measured in expired air. A study was undertaken to test the hypothesis that exhaled NO levels would be decreased in patients with SSc with PHT and to assess the utility of this measurement in discriminating between patients with and without PHT, regardless of concurrent fibrosing alveolitis. METHODS: Exhaled NO was measured with a chemiluminescence analyser in 23 patients with SSc (six with PHT, 17 subjects without) and in 67 normal individuals. Doppler echocardiography was used to assess pulmonary artery pressure in subjects with SSc, and lung function tests were performed at the same visit as NO measurements. Thin section CT scans were analysed for the presence of abnormality consistent with fibrosing alveolitis. RESULTS: Patients with SSc with PHT had a greater reduction in arterial oxygen tension (PaO2) and carbon monoxide gas transfer (TLCO) than patients with SSc without PHT. Exhaled NO was significantly higher in patients with SSc without PHT than in normal individuals, and was significantly decreased in patients with SSc with PHT (mean (SD) 20 (6) ppb) compared with 149 (19) ppb in those with SSc without PHT (mean difference 129 (95% CI 112 to 146) ppb) and 80 (7) ppb in normal individuals (mean difference 60 (95% CI 54 to 66) ppb). CONCLUSION: Exhaled NO is decreased in patients with SSc with PHT compared with both normal individuals and patients with SSc without PHT.