Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis
Conditions
Brief summary
Study of progression of fibrosis in ILD
Detailed description
The overall aims of this study are * Identify biomarkers and gene expression profiles that determine progressive fibrotic lung disease regardless of aetiology * To prospectively assess biomarkers which predict progressive fibrosis in patients with fibrosing lung disease of alternate aetiology, including RA-UIP, Asbestosis, Chronic Hypersensitivity Pneumonitis and Unclassifiable fibrotic lung disease * Investigate genetic associations and epigenetic modifications which affect fibrotic disease severity and progression * Prospectively evaluate longitudinal disease behaviour in patients with non IPF-fibrotic lung diseases with a view to developing composite clinical end-points for subsequent use in intervention studies in patients
Interventions
PROCEDUREOptional Bronchoscopy
Patients can decide to have an optional bronchoscopy so that samples can be taken for research up to three months from baseline.
OTHERQuality of Life Questionnaires
MRC Dyspnoea, SPARC, KBILD and EQ-5D-5L will be administered at baseline, 3 months, 12 months and 24 months,
a 40ml research blood sample to be taken at baseline, 3 months, 12 months and 24 months.
OTHERHome Hand Held Spirometry
Patients will download an app and are given a small hand held device to record their own spirometry at home. This is blinded for the first three months of the study and then requested a week before and a week after the three follow up points (3m, 12m, 24m)
Sponsors
Nottingham University Hospitals NHS Trust
University of Nottingham
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Male or female aged ≥ 18 years old * Able and willing to give written informed consent * Recently diagnosed \[defined as diagnostic CT scan or surgical lung biopsy (if applicable) \>1st May 2017\] * An MDT diagnosis of fibrotic interstitial lung disease (reticulation, traction +/- honeycombing) Sub Groups * Rheumatoid arthritis (rheumatologist diagnosed with anti-CCP antibodies and/or Rheumatoid Factor positive) * Asbestosis (appropriate occupational history and radiological evidence of asbestos exposure) * Chronic HP in accordance with consensus criteria (appropriate exposure history, radiological features +/- avian and fungal precipitins) * Unclassifiable fibrotic lung disease (fibrotic lung disease otherwise unclassifiable despite extensive clinical and radiological examination) * IPF in accordance with consensus criteria (ATS/ERS/JRS/ALAT guidelines) as controls
Exclusion criteria
* Participating in an interventional clinic trial * Asymptomatic Interstitial Lung Abnormalities (ILA) and normal lung function. * Change in clinical phenotype from initial radiological diagnosis to screening * Acute Hypersensitivity Pneumonitis. * Participants who do not possess a smartphone or have a valid email address (necessary for the home FVC readings)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Overall Survival | 10 years | All patients will be tagged at the central NHS registry in order to provide mortality data. For this reason we will need to keep our datasets active for up to 10 years to allow a complete mortality analysis. |
| Disease Progression | Within 12 months | Disease progression defined as \>10% relative decline in FVC (using either hospital spirometry or home hand held spirometry) or death within 12 months. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Serum and Plasma Biomarkers | Baseline, 3 months, 12 months, 24 months | SPD, MUC16, CA199, Nordic Neoepitopes |
Other
| Measure | Time frame | Description |
|---|---|---|
| DLco | Baseline, 3 months, 12 months, 24 months | Diffusing Capacity of the Lung for Carbon Monoxide |
| Quality of Life Questionnaires | Baseline, 3 months, 12 months, 24 months. | Assessment of how the patients well-being may be affected over time by their interstitial lung disease |
| Domicillary Spirometry | Daily for the first 3 months of study then at 2 week periods around time of planned follow up | Change in home handheld spirometry values from baseline to 12 weeks |
Countries
United Kingdom
Contacts
Primary ContactProf Gisli Jenkins
Backup ContactLucy Howard
Outcome results
None listed