The Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS): description of the protocol for a multicentre prospective observational cohort study identifying biomarkers of progressive fibrotic lung disease.
Introduction
The Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS) is a multicentre, prospective, observational cohort study. The aims of this study are to identify genetic, serum and other biomarkers that may identify specific molecular mechanisms, reflecting disease endotypes that are shared among patients with progressive pulmonary fibrosis regardless of aetiology. Furthermore, it is anticipated that these biomarkers will help predict fibrotic activity that may identify patterns of disease behaviour with greater accuracy than current clinical phenotyping.


Methods and analysis
200 participants with the multidisciplinary team confirmed fibrotic lung disease (50 each of rheumatoid-interstitial lung disease (ILD), asbestosis, chronic hypersensitivity pneumonitis and unclassifiable ILD) and 50 idiopathic pulmonary fibrosis participants, recruited as positive controls, will be followed up for 2 years. Participants will have blood samples, lung function tests, quality of life questionnaires and a subgroup will be offered bronchoscopy. Participants will also be given the option of undertaking blinded home handheld spirometry for the first 3 months of the study. The primary end point will be identification of a biomarker that predicts disease progression, defined as 10% relative change in forced vital capacity (FVC) or death at 12 months.


Ethics and dissemination
The trial has received ethical approval from the National Research Ethics Committee Nottingham (18/EM/0139). All participants must provide written informed consent. The trial will be overseen by the INJUSTIS steering group that will include a patient representative, and an independent chairperson. The results from this study will be submitted for publication in peer-reviewed journals and disseminated at regional and national conferences.


Trial registration number
NCT03670576.
['Khan|Fasihul|F|0000-0002-0796-5724', 'Stewart|Iain|I|0000-0002-1340-2688', 'Howard|Lucy|L|', 'McKeever|Tricia M|TM|', 'Jones|Steve|S|', 'Hearson|Glenn|G|', 'Braybrooke|Rebecca|R|', 'Edwards|Colin|C|', 'Jenkins|Gisli|G|', 'Saini|Gauri|G|']
D000328:Adult D000542:Alveolitis, Extrinsic Allergic D001195:Asbestosis D015415:Biomarkers D015331:Cohort Studies D003937:Diagnosis, Differential D005260:Female D006801:Humans D054990:Idiopathic Pulmonary Fibrosis D008297:Male D015337:Multicenter Studies as Topic D064887:Observational Studies as Topic D011446:Prospective Studies D011788:Quality of Life D013147:Spirometry D055815:Young Adult
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