Reduction of pulmonary pressure values after PGE1 infusion in sclerodermic patients with pulmonary involvment

Pulmonary involvement is a common finding in progressive systemic sclerosis, a generalized autoimmune disorder with an abnormal interstitial collagen accumulation and deterioration of small arteries and capillary vessels. In SS her's an evidence of abnormal vascular tone regulation that is evident in the lung as an increase of arterial pulmonary pressure. Aim of this study was to assess acute effect of PGE1 administration on pulmonary vascular bed-flows affected by sclerodermic alterations evaluating the response of pulmonary arterial pressure and exhaled NO to prostaglandin administration. We studied 10 female normotensive patients (age 60±2 yr.) with systemic sclerosis and pulmonary involvement (P) documented with high resolution CT with no evidence or clinical history of cardiovascular or chronic pulmonary disease The data obtained were compared with 10 sclerodermic patients (age 58±8 yr.) without pulmonary involvement (NP). All the patients underwent infusion of PGE1 (Alprostadil,60 mcg, 5 days a week for 4 weeks). An echocardiographic evaluation was performed at baseline (B), acutely post-treatment (A) and after 4 weeks stopping therapy (S) to evaluate Right Ventricular Systolic Pressure (RVSP). We also measured left and right ventricle telediastolic and systolic diameters and left ventricular mass were calculated. We also measured transmitralic flows to evaluate diastolic function and the left ventricular ejection fraction (EF%). At the same times we measured exhaled NO concentrations (ppb) with a chemi-luminescence NO gas analyzer device. (·p<0.05 A1 vs B1;∞p<0.05 S1 vs A1; °p<0.05 B2 vs B1 See the table at the bottom). Our data showed that P patients had increased RVSP values and lower NO concentrations in exhaled air compared to NP. PGE1 infusion is accompanied in these patients by a significant decrease in RVSP values and by a concomitant increase in exhaled NO concentration which both return to B conditions in follow up evaluations. A similar acute increasing trend in NO concentrations with RVSP decrease was observed in NP although it did not reached statistical significance. In conclusion PGE1 infusion may have a favorable effect on pulmunary vassels flows due to an increased of NO sensitivity and production.