It's Not JUST Idiopathic Pulmonary Fibrosis Study

Study Overview
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Brief Summary
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Study of progression of fibrosis in ILD

Detailed Description
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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

Official Title
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It's Not JUST Idiopathic Pulmonary Fibrosis Study

Conditions
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Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis

Intervention / Treatment
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* Procedure: Optional Bronchoscopy
* Other: Quality of Life Questionnaires
* Other: Blood Samples for Biomarkers
* Other: Home Hand Held Spirometry


Participation Criteria
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Eligibility Criteria
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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)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Case<br>A diagnosis of Fibrotic Lung disease classified in 4 categories, RA-UIP, Asbestosis, Chronic HP and Unclassifiable as agreed by an ILD MDT consensus panel. | Procedure: Optional Bronchoscopy<br>* Patients can decide to have an optional bronchoscopy so that samples can be taken for research up to three months from baseline.<br>Other: Quality of Life Questionnaires<br>* MRC Dyspnoea, SPARC, KBILD and EQ-5D-5L will be administered at baseline, 3 months, 12 months and 24 months,<br>Other: Blood Samples for Biomarkers<br>* a 40ml research blood sample to be taken at baseline, 3 months, 12 months and 24 months.<br>Other: Home Hand Held Spirometry<br>* 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)<br>|
| Control<br>Positive control will be frequency matched to cases of ILD and will be people in secondary care who have an MDT diagnosis of Definite IPF. | Procedure: Optional Bronchoscopy<br>* Patients can decide to have an optional bronchoscopy so that samples can be taken for research up to three months from baseline.<br>Other: Quality of Life Questionnaires<br>* MRC Dyspnoea, SPARC, KBILD and EQ-5D-5L will be administered at baseline, 3 months, 12 months and 24 months,<br>Other: Blood Samples for Biomarkers<br>* a 40ml research blood sample to be taken at baseline, 3 months, 12 months and 24 months.<br>Other: Home Hand Held Spirometry<br>* 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)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Disease Progression | Disease progression defined as >10% relative decline in FVC (using either hospital spirometry or home hand held spirometry) or death within 12 months. | Within 12 months | 
| Overall Survival | 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. | 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Serum and Plasma Biomarkers | SPD, MUC16, CA199, Nordic Neoepitopes | Baseline, 3 months, 12 months, 24 months |