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American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927815


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.

American Journal of Respiratory and Critical Care Medicine ; 203(9), 2021.
Article in English | EMBASE | ID: covidwho-1277081


RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing, interstitial pneumonia which ultimately leads to an irreversible loss of lung function and respiratory compromise. The anti-fibrotic agents, pirfenidone and nintedanib have been shown to slow the rate of decline in forced vital capacity (FVC) but, neither treatment halts disease progression. Pentraxin-2 plays important biologically relevant roles in wound repair and prevention of fibrosis. Pentraxin-2, inhibits monocyte differentiation into pro-fibrotic fibrocytes and pro-inflammatory macrophages. Plasma pentraxin-2 concentrations are reduced in patients with IPF and correlate with disease severity. Recombinant human pentraxin-2 (rhPTX-2;also known as PRM-151) was evaluated for its therapeutic potential within a phase II trial (NCT02550873). This trial demonstrated statistically significant and clinically meaningful outcomes of rhPTX-2 treatment in patients with IPF. Here we report the phase III study design to further evaluate these findings. METHODS: STARSCAPE (NCT04552899) is a phase III, multi-center, randomized, double-blind, placebo controlled trial. 658 patients with IPF will be randomized (1:1) to receive either intravenous rhPTX-2 or matching placebo administered on Days 1, 3, 5 and every 4 weeks thereafter through 48 weeks. The primary endpoint is absolute change from baseline to Week 52 in FVC [mL]. The key secondary endpoint is absolute change from baseline to Week 52 in 6-minute walk distance. Eligible patients are 40-85 years, with a documented diagnosis of IPF confirmed centrally by high resolution computed tomography scan (and lung biopsy if available). Patients must demonstrate FVC ≥ 45%, FEV1/FVC ratio > 0.70 and DLCO ≥ 30% and ≤ 90% during screening. Patients are permitted to take background therapy with nintedanib or pirfenidone. Initiating a global phase III trial during the COVID-19 pandemic brings unique and unprecedented challenges. A large number of countries and sites will be included in order to mitigate potential regional recruitment challenges that may arise during the pandemic. In addition, SARS-CoV-2 serology testing will be conducted to allow exploratory analyses on the impact of COVID-19 on lung function parameters in patients with IPF. CONCLUSIONS: rhPTX-2 has demonstrated preliminary evidence of clinical efficacy on top of approved standard of care. The phase III STARSCAPE trial aims to confirm the therapeutic potential of rhPTX-2 through evaluation of a broad range of efficacy, safety, quality of life, pharmacokinetic and biomarker assessments over 52 weeks. Patients that complete this 52-week trial may be eligible to enroll into an open label extension trial.