Conditions: Idiopathic Pulmonary Fibrosis Pulmonary Hypertension
Keywords: fibrosis, pulmonary hypertension, pulmonary exercise, aerobic
Allocation: NA | Intervention model: SINGLE_GROUP
Enrollment: 1
Healthy volunteers: no

Brief summary:
The purpose of this study is to establish single-dose tolerability of inhaled treprostinil sodium in idiopathic pulmonary fibrosis (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 in inhaled treprostinil sodium in this population.

Detailed description:
This is Phase 2, multi-center, open-label, four-cohort study in subjects with pulmonary hypertension (PH) associated with idiopathic pulmonary fibrosis (IPF). Each cohort of four subjects will receive a single dose of inhaled treprostinil sodium. Cohorts will be enrolled sequentially, starting with the lowest dose of 18 mcg. Each cohort escalate by 18 mcg increments, resulting in four cohorts of 18, 36, 54, and 72 mcg doses. Decisions to escalate to the next dose cohort will be based upon the data from the previous completed lower dose cohort of four subjects. Approximately 16 subjects are expected to receive study drug,and approximately four center in the United States with expertise in IPF will participate in this study.

Arms:

Interventions: intervention 1: Treprostinil sodium for inhalation
Intervention descriptions: intervention 1: Administration of inhaled treprostinil sodium 0.6 mg/ml in 3mL ampoules Duration of Treatment: single dose

Dose:

* Cohort 1: dosed at 3 breaths (18 mcg)
* Cohort 2: dosed at 6 breaths (36 mcg)
* Cohort 3: dosed at 9 breaths (54 mcg)
* Cohort 4: dosed at 12 breaths (72 mcg)

The decision to advance to the next cohort will be made after review of all safety information including vital signs, physical examination, clinical laboratory tests, ECGs, and adverse events.

Protocol document: