Idiopathic Pulmonary Fibrosis
Conditions
Keywords
Idiopathic Pulmonary Fibrosis, IPF, Lung Fibrosis, Biomarkers
Brief summary
The overall aim of this study is to develop a test that predicts the prognosis of IPF (Idiopathic Pulmonary Fibrosis) and which could be used to determine whether new treatments for IPF are likely to work.
Detailed description
The overall objectives of this study are * Discover and validate novel biomarkers and gene expression profiles for use in subsequent intervention studies in patients with IPF * To prospectively validate a panel of previously published biomarkers in patients with well characterized idiopathic fibrosing lung disease * Investigate genetic associations and epigenetic modifications which affect disease severity and progression * Prospectively evaluate longitudinal disease behaviour in patients with IPF and NSIP with a view to developing composite clinical end-points for subsequent use in intervention studies in patients with IPF Biomarkers that can be used for the following purposes will be identified: * Identify patients (Diagnostic)(e.g. discriminate between health and disease) * Correlate with disease severity (extent of disease, staging of disease) * Predict clinical progression (Prognostic)(stable vs progressive disease) * Track response to therapy (Therapeutic response)- Predict response to known efficacious treatments & Correlates with changes in clinical endpoints/mortality/quality of life * Predict risk of exacerbations (could be used to prevent exacerbations or reduce their severity) * Correlate with complications and/or comorbidities (e.g. biomarkers of Pulmonary Arterial Hypertension, Gastro Oesophageal Reflux in IPF, etc)
Interventions
None listed
Sponsors
Medical Research Council
GlaxoSmithKline
CRAFT Consortium
McMaster University
University of Nottingham
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 85 Years
Healthy volunteers
No
Inclusion criteria
A diagnosis of IPF using the consensus criteria (32)and Non Specific Interstitial Pneumonia. * Between the age group 18-85 years. * Sub classified into Mild (TLCO\>60), Moderate (TLCO 40-60), Severe (TLCO\<40). * People who volunteer to undergo a bronchoscopy for research
Exclusion criteria
* People who do not have IPF/NSIP (i.e. Hypersensitivity Pneumonitis, Sarcoidosis) * People who cannot give informed consent. * People who are being considered for bronchoscopy, any contra-indication to undergoing this procedure as set out in the British Thoracic Society guidelines (Thorax 2001; 56: suppl I: i1-i21). These will be part of the study but not undergo the Broncho Alveolar Lavage.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Discover biomarkers in IPF | 36 months | * Discover and validate novel biomarkers for use in subsequent intervention studies in patients with IPF * To prospectively validate a panel of previously published biomarkers in patients with well characterized idiopathic fibrosing lung disease * Investigate genetic associations and epigenetic modifications which affect disease severity and progression |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Survival from Pulmonary fibrosis. | 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. |
Countries
United Kingdom
Outcome results
None listed