About The INJUSTIS Study
INJUSTIS is a prospective multicentre observational cohort study being managed through the Nottingham Respiratory Research Unit and funded by the National Institute of Health Research (NIHR) through the Nottingham Biomedical Research Centre and an NIHR Professorship (RGJ).
Interstitial lung disease (ILD) are a group of immunoinflammatory and fibrotic diseases of the lung parenchyma. In a substantial number of patients there is progressive fibrosis of the alveoli and interstitium leading to increasing disability and death. Establishing the aetiology of these fibrotic lung diseases is often a clinical challenge and the relevance of aetiology to disease behaviour remains controversial. The best characterised fibrotic ILD is Idiopathic pulmonary fibrosis (IPF), a devastating chronic progressive disease of unknown aetiology. Other conditions characterised by progressive pulmonary fibrosis include asbestosis, chronic hypersensitivity pneumonitis (HP), rheumatoid arthritis associated interstitial lung disease (RA-ILD), and unclassifiable interstitial lung disease. In a significant proportion of patients, the progression of these related conditions is also remorseless, with recent studies highlighting phenotypic and molecular similarities across a range of ILDs, raising the possibility of shared targetable mechanisms across diseases.
To explore this hypothesis, the INJUSTIS study will recruit a clinical cohort comprising of ILD subgroups, to explore genetic, serum and clinical biomarkers that may distinguish progressive fibrosing lung disease phenotypes regardless of aetiology. This may then eventually enable therapeutics targeting specific mechanisms of disease rather than clinical phenotypes of disease.
200 participants with recently diagnosed (within 18 months of study start date) fibrotic lung disease (50 each of Rheumatoid-ILD, Asbestosis, Chronic Hypersensitivity Pneumonitis and Unclassifiable ILD) and 50 IPF participants as positive controls will be recruited from ILD clinics across the UK. A subgroup of patients will be offered home-hand spirometry and asked to perform daily blinded FVC readings for 3 months.
The trial is being overseen by the INJUSTIS steering group consisting of the Chief Investigator, centre manager, research officer, research fellow, statistician, patient representatives (Action for Pulmonary Fibrosis) and an independent chairperson. The committee meets every four months