Arterial endothelin-1 in interstitial lung disease patients with pulmonary hypertension.

The aim of this study was to evaluate the plasma arterial levels of Endothelin-1 (ET-1) and their relationship with hypoxia and pulmonary hypertension (PH) in patients with interstitial lung disease (ILD). Incremental cycle ergometry was performed in all patients up to maximal capacity. ET-1 levels during exercise (2.2 +/- 0.36 pgr/ml) were significantly higher than at rest (1.73 +/- 0.37 pgr/ml) (p < 0.001). ET-1 levels were also significantly correlated with arterial oxygen (PaO2) (r = -0.935, p < 0.001) and mean pulmonary arterial pressure (Ppa) (r = 0.657, p < 0.001). Increased pulmonary and peripheral blood levels of ET-1 have been described in, and postulated to contribute to, the pathophysiology of several lung diseases. In agreement with this, in the current study, the plasma arterial levels of ET-1 were also found to be significantly elevated in patients with various interstitial lung disorders during exercise, especially in those with severe hypoxia and pulmonary hypertension.