Inhaled iloprost as a bridge to transplant in advanced pulmonary fibrosis and secondary pulmonary hypertension

Abstract

The development of secondary pulmonary hypertension is a poor prognostic factor in patients with pulmonary fibrosis. An acute challenge of inhaled iloprost, a chemically stable derivative of prostacyclin, has previously been shown to cause selective pulmonary vasodilatation in secondary pulmonary hypertension. We assessed its short-term efficacy in patients with advanced pulmonary fibrosis and secondary pulmonary hypertension as a bridge to transplantation. We studied seven patients (4 with UIP, and 3 with advanced sarcoidosis) all in New York Heart Association functional class IV. Iloprost was nebulized using a Schill multisonic nebulizer, and administered 3 hourly in increasing daily doses to a maintenance of 90mcg/day. The effects of inhaled iloprost were assessed by echocardiography, shuttle walk test and right heart catheterization (n 2). Inhalation of iloprost was well tolerated, and administered for a mean (SEM) of 2.4 (0.9) months. Two of 4 UIP patients and 1 of 2 sarcoid patients died during the follow up. There was a fall in pulmonary artery pressure, 73mmHg (19) before iloprost, versus 40mmHg (9) after iloprost therapy (p 0.26). There was a non significant improvement in pulmonary artery pressure when assessed by echo, 60mmHg (8) before versus 48mmHg (7) after iloprost therapy (p 0.28). Shuttle walk distance improved, but not significantly, 86m (34) before, versus 104m (48) after iloprost therapy (p 0.77). Inhaled iloprost therapy with terminal pulmonary fibrosis may only benefit selected (sarcoid) patients.