Prospective Study of Fibrosis In the Lung Endpoints (PROFILE - Central England)

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)

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.