Remote Early Identification of Disease Progression in Connective Tissue Disease related Interstitial Lung Disease (CTD-ILD) using Digital Patient Empowered Monitoring

Abstract

Interstitial lung disease (ILD) is an increasingly recognised complication in connective tissue diseases (CTD). Early detection of progressive ILD amongst patients with CTD is essential for administration of treatment in a timely manner. We aim to determine if domiciliary smartphone app-based spirometer monitoring can detect early progressive fibrosing ILD in CTD-ILD resulting in earlier treatment ultimately leading to a reduction in mortality and morbidity. Participants with CTD-ILD and with IPF were recruited and will be followed prospectively over 12 months. At baseline, participants underwent pulmonary function testing (PFT), high resolution CT thorax (HRCT), blood sampling for laboratory testing and genotyping, 6-minute walk test (6MWT), echocardiogram and Quality of Life (QoL) Questionnaires. 6-monthly PFTs, 6MWT and QoL Questionnaires will be performed. Echocardiogram and HRCT will be repeated in 12 months. Participants also use a handheld spirometer linked to a real-time electronic health journal generated via a smart-phone app. To date, 27 patients with CTD-ILD and 4 with IPF have been recruited. Median age was 65 years and 45.2% were males. 64.5% were using home spirometry monitoring regularly. Preliminary data showed median FVC of 1.96L and 2.63L in the CTD-ILD and IPF cohorts respectively. This is a novel approach to detect patients with rapidly progressive ILD, identifying those who would benefit most from treatment thereby improving quality of life and life expectancy.