Pulmonary Fibrosis
Conditions
Brief summary
The purpose of this study is to determine if measurements of active collagen deposition using \[68Ga\]CBP8 positron emission tomography (PET) and tissue injury using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) can predict an individual patient's pace of disease progression in non-idiopathic pulmonary fibrosis interstitial lung disease (non-IPF ILD) and identify which individuals will develop progressive pulmonary fibrosis.
Detailed description
60 participants with non-idiopathic pulmonary fibrosis interstitial lung disease (non-IPF ILD) on stable dose immunosuppression treatment will be enrolled. Participants will undergo combined \[68Ga\]CBP8 positron emission tomography (PET) and dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) at baseline. The investigators will compare the ability of PET and MRI measurements performed over the whole lung and within regions of interest to identify participants who subsequently develop progressive pulmonary fibrosis as determined by changes in pulmonary function testing, quantitative fibrosis on high-resolution computed tomography, and respiratory symptoms over 24 months. The investigators will also test whether combining the PET and MRI measurements results in more accurate prediction of progression than either modality alone.
Interventions
DRUG[68Ga]CBP8
Participants will receive a single intravenous injection of up to 350 MBq of \[68Ga\]CBP8
Participants will receive a single intravenous injection of 0.05 mmol/kg gadoterate meglumine during DCE-MRI
Sponsors
Peter Caravan
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
1. Age 18-80 with a diagnosis of chronic hypersensitivity pneumonitis, connective tissue-associated ILD (due to rheumatoid arthritis, systemic sclerosis, mixed connective tissue disease), or undifferentiated ILD. 2. On stable dose immunosuppression treatment (with prednisone, mycophenolate mofetil, and/or rituximab) for at least 3 months. 3. Pulmonary fibrosis, defined as honeycombing, traction bronchiectasis, or reticular opacities on HRCT performed within 1 year to or at Visit 1. 4. FVC of \>/= 45% and DLCO \>/= 25% predicted on PFTs performed at Visit 1.
Exclusion criteria
1. Current or prior exposure to FDA approved anti-fibrotic therapy. 2. Extent of emphysema greater than extent of fibrosis. 3. Pregnancy or plans to become pregnant at baseline or during follow-up. 4. Contraindications to MRI. 5. Contraindications to receiving gadolinium-based contrast agents. 6. Research-related radiation exposure exceeds 50 mSv in the prior year. 7. Estimated glomerular filtration rate (eGFR) \< 30 mL/min (only for individuals with a history of chronic kidney disease). 8. Clinically significant PH defined by use of pulmonary vasodilatory therapy. 9. Respiratory infection within the prior 6 weeks. 10. Smoking of any kind within the prior 6 months.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Development of progressive pulmonary fibrosis | Up to 24 months | Defined by the 2022 ATS guideline definition of progressive pulmonary fibrosis (PPF) which defines PPF as satisfying 2 of 3 criteria within 12 months: worsening symptoms, physiologic progression (absolute decline in FVC ≥ 5% or absolute decline in DLCO ≥ 10%), or radiologic evidence of disease progression. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Decline of forced vital capacity (FVC) ≥ 5% from baseline | Up to 24 months | FVC will be measured at baseline, 6, 12, 18, and 24 months |
| Decline of forced vital capacity (FVC) ≥10% from baseline | Up to 24 months | FVC will be measured at baseline, 6, 12, 18, and 24 months |
| Decline of diffusing capacity for carbon monoxide (DLCO) ≥15% from baseline | Up to 24 months | DLCO will be measured at baseline, 6, 12, 18, and 24 months |
Countries
United States
Contacts
Primary ContactSydney Montesi, MD
Backup ContactCeanna Kalaria
Outcome results
None listed