PURPOSE: Sarcoidosis associated pulmonary hypertension (SAPAH) can lead to significant dyspnea which may be unresponsive to immunosuppressive therapy. Recent case reports have demonstrated that SAPAH may respond to vasodilator therapy, including intravenous epoprostenol.
METHODS: We have initiated a prospective open-label study to evaluate the benefit of inhaled iloprost 2.5 to 5.0 mcg six times a day in patients with SAPAH. Right heart catheterization is performed prior to and after 16 weeks of therapy. Six minute walk distance (6MWD) is determined prior to and monthly throughout the study.
RESULTS: To date, 12 sarcoidosis patients have been enrolled in this two center study. Patient demographics include 10 African Americans, 6 women, and age 52 (41-64) years (Median (Range)). Of these 12, 7 were classified as WHO class 3, the others WHO class 2. The baseline hemodynamic values include PA systolic pressure was 59 (45-70) Torr, PA mean= 36 (27-50) Torr, cardiac output= 5.5 (3.5-9.5) liters/min. Baseline 6 minute walk distance (6MWD) was 335 (23-478) m. Follow up studies have been performed on four patients. Three patients had a documented fall in PA mean (1.7, 6.7, and 13.3 Torr) while one patient had a rise in PA mean (4.3 Torr). Three patients had an increase in their 6MWD (30, 46, and 53 m), while one patient had a fall in 6MWD, but had a decrease in her PA mean of 6.7 Torr. To date, two patients discontinued drug because of severe cough.
CONCLUSION: SAPAH can be a significant reason for reduced 6MWD. Inhaled iloprost was effective in improving hemodynamics and 6MWD in some patients with SAPAH.
CLINICAL IMPLICATIONS: Reduction in 6MWD due to SAPAH may respond to pulmonary vasodilator therapy, including inhaled iloprost.
DISCLOSURE: Robert Baughman, Grant monies (from industry related sources) From Actelion to support this trial; Consultant fee, speaker bureau, advisory committee, etc. Actelion speaker bureau; Product/procedure/technique that is considered research and is NOT yet approved for any purpose. Iloprost is not approved for SAPAH