Long-term effects of intermittent Iloprost infusion on pulmonary arterial pressure in connective tissue disease

BackgroundIntravenous periodic Iloprost is proven effective in the treatment of Raynaud phenomenon (RP) related to connective tissue disorder (CTD). It's well known that synthetic prostaglandins are effective drugs for the treatment of pulmonary arterial hypertension (PAH), and that PAH is frequently associated with CTD.ObjectiveThe aim of the study is to evaluate in the chronic effect of cyclic intravenous Iloprost on pulmonary arterial pressure.MethodsWe studied 17 consecutive patients with CTD (14 systemic sclerosis, 3 mixed CTD) and RP, at the entry and after at least 6 months of treatment of RP with cyclic Iloprost. On both occasions, in all patients we performed transthoracic Doppler echocardiography and we determined NT-proBNP plasma levels, NYHA functional class, 6 Minute-Walk Distance (6MWD).ResultsAt follow-up (8.2 ± 1.9 months; range 6–12) mean values of pulmonary arterial systolic pressure (PASP) significantly decreased (from 32.2 ± 9.2 to 29.2 ± 7.6 mmHg, p < 0.04) and mean values of 6MWD significantly increased (from 407.5 ± 101.5 to 448.3 ± 89.9 m, p < 0.01). Moreover, we observed a significant direct correlation between PASP and NT-proBNP values and a significant inverse correlation both between NT-proBNP and 6MWD values and between PASP and 6MWD values.ConclusionOur results suggest that cyclic intravenous Iloprost may protect against the development or worsening of PAH in patients with CTD and RP.