Australia and New Zealand Interstitial Lung Disease Registry (ANZ ILD) 2021 Update - Progress During the Pandemic

Abstract

Introduction/Aim: Interstitial lung disease (ILD) comprises a heterogeneous group of diseases affecting the lung interstitium associated with significant morbidity and mortality. The Australasian Interstitial Lung Disease Registry (AILDR) launched in 2016 with 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. Methods: Consecutive ILD patients attending 16 registered ILD centres across Australia and NZ are eligible to enrol in the AILDR. 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 30 September 2021 inclusive. Results: In total 2140 participants were enrolled from 16 sites at a mean rate of 43/month (mean age 65.8 ± 13.3 years;1185 (55.4%) male;982 (45.9%) eversmokers;mean BMI 29.4 ± 5.9 kg/m2). Baseline functional parameters demonstrated mean FVC 85.6 ± 21.7% predicted, mean DLCO 60.5 ± 19.4% predicted, and mean 6-min walk test (6MWT) distance 434.3 ± 126.5 m. 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 and older compared to all other ILD subtypes (p < 0.001). Baseline functional parameters were lowest for those with CHP, significantly lower comparable to the IPF group (p < 0.001). Highest baseline functional parameters were observed in those with sarcoidosis. Mortality data demonstrated a cumulative rate of death in year one, two, three and four of 8%, 15%, 25% and 44%, respectively. Conclusion: We demonstrate the feasibility of a bi-national ILD registry evidenced by steady recruitment despite the COVID-19 pandemic. Through a routine approach across Australasia, the AILDR aims to improve standardization of diagnosis and management of ILD patients.