Long-term therapy with inhaled iloprost in patients with pulmonary hypertension.
OBJECTIVE
To investigate the long-term safety of inhaled iloprost in patients with pulmonary hypertension (pH), including idiopathic PAH (IPAH group) and other forms of pulmonary hypertension (PHother).


RESULTS
Sixty-three patients (IPAH group, n=40, PHother n=23) were enrolled to receive inhaled iloprost either from baseline or after 3 months in a prospective, open-label 2-year study. Iloprost was inhaled 6-9 times daily with a night pause employing a jet nebulizer delivering an inhaled single dose of 4microg at the mouthpiece. In the case of side effects the single dose was reduced to 2microg. Sixty patients received at least 1 dose of inhaled iloprost. Thirty-six patients completed at least 630 days of therapy (25 IPAH, 11 PHother), 19 patients dropped out prematurely and 8 patients died (3 IPAH, 5 PHother). There were no drug-induced toxicities and only mild to moderate side effects. The most common side effects were coughing and flushing. Two-year survival was estimated at 85% (IPAH group 91%, PHother 78%). A modified analysis was performed to correct for differential drop-out. It included follow-up data from the premature discontinuations and revealed a 2-year survival of 87% [95% CI, 76%-98%] in the IPAH group while the predicted survival was 63%. The iloprost dose increased by 16% over 2 years.


CONCLUSIONS
Inhaled iloprost is well tolerated as long-term therapy and no substantial dose increase is required. Although uncontrolled, the data suggest a long-term clinical benefit from continued therapy with inhaled iloprost.
University Hospital Giessen and Marburg GmbH, Klinikstr 36, Giessen, Germany. horst.olschewski@medunigraz.at
Horst
H
Olschewski
Marius M
MM
Hoeper
Juergen
J
Behr
Ralf
R
Ewert
Andreas
A
Meyer
Mathias M
MM
Borst
Jörg
J
Winkler
Michael
M
Pfeifer
Heinrike
H
Wilkens
Hossein Ardeschir
HA
Ghofrani
Sylvia
S
Nikkho
Werner
W
Seeger
D000280:Administration, Inhalation; D000293:Adolescent; D000328:Adult; D000368:Aged; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000139:chemically induced / Q000401:mortality / Q000503:physiopathology*; D016285:Iloprost / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects / Q000502:physiology*; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects; D055815:Young Adult
D014665:Vasodilator Agents; D016285:Iloprost