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Published on: March 8, 2019
Inhaled iloprost for sarcoidosis associated pulmonary hypertension
R P Baughman1, M A Judson, E E Lower
1Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA. bob.baughman@uc.edu
Summary
Inhaled iloprost improved pulmonary hemodynamics and quality of life in some patients with sarcoidosis-associated pulmonary hypertension (SAPH). This study explored the effectiveness of inhaled prostacyclin therapy for SAPH patients.
Area of Science:
- Pulmonary Medicine
- Cardiology
- Pharmacology
Background:
- Sarcoidosis-associated pulmonary hypertension (SAPH) is a serious condition.
- Systemic prostacyclin therapy has shown some efficacy in SAPH patients.
Purpose of the Study:
- To evaluate the response rate of SAPH patients to inhaled prostacyclin, specifically iloprost.
- To assess the impact of inhaled iloprost on pulmonary hemodynamics and quality of life in SAPH.
Main Methods:
- An open-label, prospective study enrolled 22 SAPH patients without left ventricular dysfunction.
- Patients received inhaled iloprost (5 mcg) every 2-3 hours while awake.
- Evaluations included right heart catheterization, 6-minute walk tests, and quality of life questionnaires (SGRQ) at baseline and after 16 weeks.
Main Results:
- Fifteen of 22 patients completed the 16-week therapy; common discontinuations were cough and compliance issues.
- Six patients showed a ≥20% decrease in pulmonary vascular resistance (PVR), with five also having a ≥5 mm Hg reduction in mean pulmonary artery pressure.
- A significant decrease in SGRQ activity score was observed (p=0.0273), with seven patients improving by ≥4 points.
Conclusions:
- Inhaled iloprost monotherapy demonstrated potential benefits for some SAPH patients.
- Improvements were noted in pulmonary hemodynamics and quality of life, particularly the SGRQ activity score.
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