It's Not JUST Idiopathic Pulmonary Fibrosis Study

It's Not JUST Idiopathic Pulmonary Fibrosis Study

Study of progression of fibrosis in ILD

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

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)