Aerosolized iloprost in CREST syndrome related pulmonary hypertension

Objective. To assess the outcome of patients with CREST syndrome associated severe pulmonary hypertension treated by aerosolized iloprost in a noncomp arative study. Methods. Five patients with CREST syndrome associated severe pulmonary hype rtension were treated with 100 mug/day of aerosolized iloprost. New York He art Association functional class and exercise tolerance (6 min walk, test) were assessed at baseline, after one month, and then every 6 months. A righ t heart catheterization was performed at baseline in all but one patient. S ystolic pulmonary artery pressure (PAP) was measured with Doppler echocardi ography after one month and every 6 months. Results. The mean followup was 13.2 +/- 8.8 months (median 6, range 6-24). Subjective quality of life improved in all patients. NYHA functional class decreased from Class III to II in 3 patients, from Class III to I in one pa tient, and from Class IV to III in one patient. At 6 months, the distance w alked in 6 min had increased from 352 +/- 48 to 437 +/- 56 in (p = 0.06). A t one month the mean Systolic PAP was 58 +/- 13 vs 81 +/- 9 min Hg at basel ine (p = 0.04). At 6 months the mean systolic PAP was 57 +/- 13 min Hg (p = 0.06). The improvement of both clinical and hemodynamic status was maintai ned in the 2 patients treated for 2 years. Neither adverse effects nor need to increase the daily dose of iloprost were observed. One patient died of right heart failure and one patient did not experience any improvement of e xercise tolerance and hemodynamics. Conclusion. Aerosolized iloprost might be potentially useful as treatment f or CREST syndrome associated pulmonary hypertension. However, patients who could benefit from this treatment will probably have to undergo careful cri teria selection.