While there are no FDA approved therapies for the treatment of IPF, there are therapies that have been shown to be effective in ameliorating the effects of pulmonary hypertension in patients with PAH (pulmonary arterial hypertension), including inhaled iloprost. However, these therapies have not been used extensively in patients with IPF who have pulmonary hypertension as a complication. The potential benefits of treatment of pulmonary hypertension complicating IPF include: improvement in pulmonary hemodynamics, with reduction in pulmonary artery pressure and pulmonary vascular resistance, and increase in cardiac output.
