Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a bi-national prospective database

Background Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. There is increasing recognition of the need for improved epidemiological data to help determine best practice and improve standardisation of care. The Australasian ILD Registry (AILDR) is a bi-national registry of patients with all ILD subtypes designed to establish a clinically meaningful database reflecting real world practice in Australasia with an objective to improve diagnostic and treatment pathways through research and collaboration. Methods AILDR is a prospective observational registry recruiting patients attending ILD clinics at centres around Australia and New Zealand. Core and non-core data are stored on a secure server. The pilot phase was launched in 2016 consisting of four sites in Australia. Currently in its second phase a further 16 sites have been recruited, including three in New Zealand. Results A total of 1061 participants were consented during the pilot phase. Baseline data demonstrated a mean age 68.3 [+ or -] 12.5 (SD) years, mean FVC (%predicted) 79.1 [+ or -] 20.4 (SD), mean DLCO (%predicted) 58.5 [+ or -] 17.9 (SD) and nadir exertional SpO2 (%) 91 [+ or -] 6.9 (SD). Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. Baseline demographics and physiology were not significantly different across the four centres. Conclusion AILDR is an important clinical and research tool providing a platform for epidemiological data that will prove essential in promoting understanding of a rare cohort of lung disease and provide foundations for our aspiration to standardise investigation and treatment pathways of ILD across Australasia. Keywords: Interstitial lung disease, Lung fibrosis, Autoimmune disease, Clinical epidemiology, Registry