Pipeline
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrosing lung disease of unknown etiology for which there are no currently approved therapies. Some patients with IPF develop pulmonary hypertension (PH), which may contribute to their poor functional status, and which is associated with a particularly poor prognosis. Although the etiology of PH in patients with IPF has not been established, several possibilities have been suggested, including fibrotic destruction of pulmonary vasculature, local or systemic hypoxemia, pulmonary thromboembolism, cardiac dysfunction, genetic predisposition, and variable cytokine or chemokine expression. Given the association between PH and increased mortality in IPF, it has been suggested that therapy directed toward PH may benefit patients with IPF.
Lung Rx is conducting a study entitled "A Single-Dose, Dose-Escalating Study Exploring the Safety, Pharmacokinetics, and Pharmacodynamic Effects of Inhaled Treprostinil Sodium Using the Nebu-Tec OPTINEB® Inhalation Device in Patients with Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis". The purpose of this study is to establish single-dose tolerability of inhaled treprostinil sodium in IPF patients with pulmonary hypertension, and to explore the acute hemodynamic effects over a range of tolerable doses. The safety and pharmacodynamic information obtained from this study will inform the design and conduct of future studies of inhaled sodium in this population.