ABSTRACT
Introduction: Interstitial lung disease (ILD) comprises a heterogeneous group of diseases affecting the lung interstitium often associated with significant morbidity and mortality. The Australasian Interstitial Lung Disease Registry (AILDR) launched in 2016 with the concurrent aims to: a) provide a valuable resource for high quality ILD research to further understanding of ILD and b) improve care for ILD patients across Australia and NZ. Consisting initially of four pilot sites, over time the registry has expanded to 21 sites across Australasia. Methods: Consecutive ILD patients attending any of the registered ILD centres across Australia and NZ are eligible to enrol in the AILDR following provision of informed consent. Comprehensive data including demographics, ILD diagnosis, objective functional markers (baseline and subsequent tests) and treatment parameters are collected and stored on a secure online platform. We report data from the AILDR since initiation in May 2016 to 30th September 2021 inclusive. Results: In total 2140 participants were enrolled from 16 sites at a mean rate of 43/month (mean age 65.8±13.3years;1185 (55.4%) male;982 (45.9%) ever-smokers;mean BMI 29.4±5.9kg/m2). Baseline functional parameters demonstrated mean FVC 85.6±21.7% predicted, mean DLCO 60.5±19.4%predicted, and mean six-minute walk test (6MWT) distance 434.3±126.5metres. ILD diagnoses included: idiopathic pulmonary fibrosis (IPF) n=545 (30.3%), connective tissue disease associated ILD (CTD-ILD) n=326 (18.1%), chronic hypersensitivity pneumonitis (CHP) n=155 (8.6%), sarcoidosis n=120 (6.7%) and unclassifiable ILD n=190 (10.6%). Patients with IPF were more likely to be male (n=403, 73.9%, p<0.001) and older (72.6±8.3years, p<0.001) compared to all other ILD subtypes. A female predominance was observed for CHP (n=92, 59%, p=0.001) and CTD-ILD (n=206, 63%, p<0.001). Baseline functional parameters were lowest for those with CHP (FVC 76.8±22.4% predicted, DLCO 54.1±16.9% predicted), significantly lower comparable to the IPF group (FVC 84.8±19.6%predicted, DLCO 58.7±17.8%predicted, p<0.001). The highest baseline functional parameters were observed in those with sarcoidosis. Conclusion: We demonstrate the feasibility of a bi-national ILD registry evidenced by steady recruitment despite the COVID-19 pandemic. In this study, lower functional baseline parameters were detected in the CHP group suggesting priority research should be afforded to this group. Through a routine approach across Australasia, the AILDR aims to improve standardisation of diagnosis and management of ILD patients.