It's Not JUST Idiopathic Pulmonary Fibrosis Study (INJUSTIS)

University of Nottingham

Status: Unknown

Conditions: Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease

Treatments: Other: Quality of Life Questionnaires; Other: Blood Samples for Biomarkers; Procedure: Optional Bronchoscopy; Other: Home Hand Held Spirometry

Study type: Observational

Funder types: Other

Identifiers

NCT03670576

18014

Details and patient eligibility

About

Study of progression of fibrosis in ILD

Full 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

Enrollment: 250 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion and exclusion criteria

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)

Trial design

250 participants in 2 patient groups

Case

Description:

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.

Treatment:

Other: Blood Samples for Biomarkers

Procedure: Optional Bronchoscopy

Other: Home Hand Held Spirometry

Other: Quality of Life Questionnaires

Control

Description:

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.

Treatment:

Other: Blood Samples for Biomarkers

Procedure: Optional Bronchoscopy

Other: Home Hand Held Spirometry

Other: Quality of Life Questionnaires