Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension

Pulmonary hypertension has been described in sarcoidosis. It can be a significant problem, not responsive to treatment with anti-inflammatory drugs for the sarcoidosis (1;2). Inhaled iloprost has been approved for treatment of pulmonary hypertension (3). We propose to study the effectiveness of inhaled iloprost for sarcoidosis associated pulmonary hypertension (SAPAH). This is an open label trial, with patients receiving 16 weeks of therapy. Clinical and hemodynamic outcome of therapy will be assessed.

Inclusion criteria

• Patients with known sarcoidosis 17
• Age 18 or greater
• Patients with documented pulmonary hypertension with a PA mean > 25 mm as measured by cardiac catheterization within six months of entry into the study
• Patients with dyspnea
• Six minute walk distance of between 100 to 500 meters
• Patients on stable immunotherapy for their sarcoidosis, including prednisone, methotrexate, azathioprine, hydroxychloroquine, cyclophosphamide, thalidomide, and/or infliximab
• Patients able to provide written consent

Exclusion criteria

• Patients on pulmonary vasodilator drugs (flolan, remodulin, bosentan, sildenafil) in the prior 28 days (patients on stable dose of calcium channel blocker for more than 1 month prior to right heart catheterization can be continued on the calcium channel blocker)
• Patients with severe airway obstruction as defined by FEV1/FVC of less than 35%
• Patients with World Health Organization (WHO) class IV status
• Patients who are pregnant or breast feeding
• Patients with significant left ventricular dysfunction with a left ventricular ejection fraction of less than 35%
• Significant liver dysfunction not due to sarcoidosis
• Patients with severe other organ disease felt by investigators to impact survival during the course of the study
• Patients unable to perform the 6 inhalation treatments required for therapy
• Patients with < 90 mm Hg Systolic systemic blood pressure will be excluded