It's Not JUST Idiopathic Pulmonary Fibrosis Study

• Conditions: Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease
• Interventions: Procedure: Optional Bronchoscopy; Other: Quality of Life Questionnaires; Other: Blood Samples for Biomarkers; Other: Home Hand Held Spirometry

• Registration Number: NCT03670576
• Lead Sponsor: University of Nottingham

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

Study Timeline

• Study First Submitted: 2018-08-06
• Study First Submitted QC: 2018-09-12
• Study First Posted: 2018-09-13
• Study Start: 2018-11-11
• Status Verified: 2021-07
• Last Update Submitted: 2021-07-01
• Last Update Posted: 2021-07-02
• Primary Completion: 2022-11-11
• Study Completion: 2022-11-11

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 250

Inclusion Criteria

Not provided

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)

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Case	Optional Bronchoscopy	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.
Case	Quality of Life Questionnaires	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.
Case	Blood Samples for Biomarkers	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.
Control	Optional Bronchoscopy	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.
Control	Home Hand Held Spirometry	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.
Control	Quality of Life Questionnaires	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.
Control	Blood Samples for Biomarkers	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.
Case	Home Hand Held Spirometry	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.

Primary Outcome Measures

Name	Time	Method
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.
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.

Secondary Outcome Measures

Name	Time	Method
Serum and Plasma Biomarkers	Baseline, 3 months, 12 months, 24 months	SPD, MUC16, CA199, Nordic Neoepitopes

Trial Locations

Locations (24)

University Hospitals Birmingham; 🇬🇧 Birmingham, United Kingdom

Blackpool Victoria Hospital; 🇬🇧 Blackpool, United Kingdom

Nottingham University Hospitals NHS Trust; 🇬🇧 Nottingham, United Kingdom

Royal Albert and Edward Infirmary; 🇬🇧 Wigan, United Kingdom

University Hospitals Coventry and Warwickshire; 🇬🇧 Coventry, United Kingdom

Newcastle Upon Tyne NHS Foundation Trust; 🇬🇧 Newcastle, United Kingdom

University Hospital of North Tees; 🇬🇧 Stockton-on-Tees, United Kingdom

Queens Hospital Burton; 🇬🇧 Burton on Trent, Derbyshire, United Kingdom

Kings Mill Hospital; 🇬🇧 Mansfield, Nottingham, United Kingdom

Royal United Hospitals Bath NHS Trust; 🇬🇧 Bath, United Kingdom

Medway Maritime Hospital; 🇬🇧 Gillingham, United Kingdom

South Tyneside District Hospital; 🇬🇧 South Shields, United Kingdom

St Georges Hospital; 🇬🇧 London, United Kingdom

Kingston Hospital NHS Foundation Trust; 🇬🇧 London, United Kingdom

Royal Devon and Exeter Hospital; 🇬🇧 Exeter, United Kingdom

Taunton and Somerset NHS Foundation Trust; 🇬🇧 Taunton, United Kingdom

Sheffield Teaching Hospitals NHS Foundation Trust; 🇬🇧 Sheffield, United Kingdom

Southmead Hospital North Bristol; 🇬🇧 Bristol, United Kingdom

Royal Derby Hospital; 🇬🇧 Derby, United Kingdom

New Cross Hospital; 🇬🇧 Wolverhampton, United Kingdom

Worcestershire Royal Hospital; 🇬🇧 Worcester, United Kingdom

North Manchester General Hospital; 🇬🇧 Manchester, United Kingdom

Wythenshawe Hospital; 🇬🇧 Manchester, United Kingdom

Northumbria Healthcare NHS Foundation Trust; 🇬🇧 Newcastle, United Kingdom