Inhaled iloprost for sarcoidosis associated pulmonary hypertension.
BACKGROUND
Patients with sarcoidosis associated pulmonary hypertension (SAPH) have responded to systemic prostacyclin therapy.


OBJECTIVE
To determine the rate of response to inhaled prostacyclin, iloprost, in SAPH.


METHODS
Sarcoidosis patients with pulmonary hypertension and no evidence for left ventricular dysfunction were enrolled in an open label, prospective study. Patients underwent right heart catheterization and six minute walk (6MW) test. Quality of life was evaluated using several instruments, including the Saint George Respiratory Questionnaire (SGRQ). Patients received 5 mcg of inhaled iloprost every 2-3 hours while awake. After four months of therapy, patients underwent repeat cardiac catheterization, 6 MW test, and completed quality of life questionnaires.


RESULTS
Of the 22 patients enrolled, 15 completed all 16 weeks of therapy. The most common reasons for study discontinuation included drug associated cough (3 patients) and compliance with the prescribed number of treatments per day (2 patients). Six patients experienced a 20% or greater decrease in pulmonary vascular resistance (PVR) from baseline with five of these six patients also showing > or = 5 mm Hg reduction in PA mean. Although three patients improved the 6MW distance by at least 30 meters, only one had a decrease in PVR. At 16 weeks a significant decrease was reported in the SGRQ activity score (p = 0.0273), with seven patients having a 4 point or greater decrease.


CONCLUSIONS
Inhaled iloprost as monotherapy was associated with an improvement in pulmonary hemodynamics and quality of life as assessed by the SGRQ activity score in some sarcoidosis patients with SAPH.
Baughman
R P
RP
Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA. bob.baughman@uc.edu
Judson
M A
MA
Lower
E E
EE
Highland
K
K
Kwon
S
S
Craft
N
N
Engel
P J
PJ
D000280:Administration, Inhalation; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000139:chemically induced* / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011669:Pulmonary Wedge Pressure / Q000187:drug effects*; D011788:Quality of Life; D017565:Sarcoidosis, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D011795:Surveys and Questionnaires; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000009:adverse effects*
D014665:Vasodilator Agents; D016285:Iloprost