Treprostinil Therapy For Patients With Interstitial Lung Disease And Severe Pulmonary Arterial Hypertension
Condition: Pulmonary Arterial Hypertension; Interstitial Lung Disease; Idiopathic Pulmonary Fibrosis
MeSH terms: Lung Diseases; Pulmonary Fibrosis; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Idiopathic Pulmonary Fibrosis; Lung Diseases, Interstitial; Hypertension; Fibrosis
Intervention: Treprostinil
Intervention description: For both SQ and IV routes, treprostinil will be started in the hospital at 1ng/kg/min and titrated up by 1ng/kg/min every 1-3 days as tolerated
Intervention MeSH terms: Treprostinil
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 15.0
Sex: All
Healthy volunteers: No
Keywords: pulmonary hypertension; pulmonary fibrosis; interstitial lung disease

Summary:

      Our hypothesis is that IV or SQ Treprostinil can improve 6 minute walk distance, hemodynamics
      and quality of life in patients with interstitial lung disease and severe secondary pulmonary
      arterial hypertension.
    

Eligibility criteria:

        Inclusion Criteria:

        Eligible subjects must have IPF and severe pulmonary arterial hypertension (PAH) documented
        on standard of care right-heart catheterization (RHC) and planned to receive therapy with
        treprostinil as recommended by the treating physician.

          1. All subjects must have high resolution CT scan (HRCT) diagnostic of IPF (performed as
             part of standard of care evaluation) or if available, biopsy proven histological usual
             interstitial pneumonia (UIP).

          2. Severe pulmonary arterial hypertension defined as a resting mean pulmonary artery
             pressure (mPAP) > 35 mm Hg; AND pulmonary vascular resistance (PVR) > 3 woods-units;
             AND pulmonary capillary wedge pressure (PCWP) < 18 mm Hg by right-heart
             catheterization (RHC) performed as part of standard of care evaluation.

          3. All subjects must be planned to receive treprostinil therapy as recommended by their
             treating physician.

        Exclusion Criteria:

          1. Acute or chronic impairment other than dyspnea (e.g. angina pectoris, intermittent
             claudication) limiting the ability to perform standard of care six-minute walk tests
             (6MWT).

          2. Six-minute walk distance (6MWD) < 50 meters at screening or baseline standard of care
             evaluations

          3. Standard of care pulmonary function test (PFT) showing forced expiratory volume in one
             second (FEV1)/ forced vital capacity (FVC) ratio < 0.65

          4. Standard of care pulmonary function test (PFT) showing a residual volume >120%
             predicted

          5. Standard of care high-resolution chest computed tomography (HRCT) showing emphysema
             extent > 30%

          6. Any investigational therapy as part of a clinical trial for any indication with 30
             days before screening

          7. Change in dose of treatment for IPF - investigational agent (gamma interferon-1b,
             pirfenidone, etanercept, and any other investigational agent intended to treat IPF),
             corticosteroids, or cytotoxic agents, within 30 days before screening. That is,
             subjects can be on any of these agents provided the dose is stable for at least 30
             days prior to enrollment.

          8. Current treatment for pulmonary hypertension with other prostaglandins (epoprostenol
             or iloprost)

          9. Change in dose of treatment for PAH - (bosentan, sitaxsentan, ambrisentan, tadalafil,
             sildenafil, vardenafil, calcium channel blockers, nitrates, digitalis), within 30 days
             before screening. That is, subjects can be on any of these agents provided the dose is
             stable for at least 30 days prior to enrollment

         10. Pulmonary rehabilitation initiated within 30 days of baseline.