BACKGROUND AND AIMS:Real-world evaluation of clinical characteristics and treatment profile of patients with idiopathic pulmonary fibrosis (IPF) is needed to establish areas for improvement according to evidence-based recommendations. This study evaluated the clinical characteristics and treatment profile of patients with IPF from the Australasian ILD Registry (AILDR), comparing them to the Australian IPF Registry (AIPFR) cohort, to identify differences in baseline characteristics among IPF patients treated with antifibrotics and immunosuppressants. METHODS:Consecutive patients with IPF enrolled between May 2016 and December 2023 from the AILDR and data of patients enrolled in the AIPFR between 2012 and 2016 were included. RESULTS:A total of 842 and 647 patients with IPF were included from the AILDR and the AIPFR respectively. Both cohorts were male predominant (AILDR: 72.0%; AIPFR: 67.7%) of similar mean body mass index (AILDR: 29.2 ± 4.9 kg/m2; AIPFR: 28.7 ± 4.8 kg/m2). With regard to disease-targeted treatment, 572 (67.9%) and 108 (12.8%) participants of the AILDR cohort were on antifibrotic medications and immunosuppressants respectively. There were a total of 35.6% (n = 300) IPF patients on pirfenidone and 40.4% (n = 340) on nintedanib in AILDR. Only 146 (23%) of the AIPFR cohort were receiving antifibrotics. Of newly registered patients in 2016 (n = 18), 2020 (n = 71) and 2023 (n = 151), 50.0%, 18.3% and 15.2% respectively had received immunosuppressive therapy. CONCLUSION:Baseline demographics from the IPF cohorts for both AILDR and AIPFR are largely comparable, with increasing antifibrotic and decreasing use of immunosuppressants since 2016. Immunosuppressive therapy in IPF remains relatively higher than expected despite guideline recommendations.

BACKGROUND:Cough is a common symptom in interstitial lung disease (ILD), often leading to treatment dissatisfaction for patients and physicians.AIM:To identify the prevalence and subjective adequacy of control of cough in patients with ILD.METHODS:A cross-sectional study of patients with ILD attending a tertiary ILD clinic in Perth was undertaken using a pre-designed questionnaire that patients were invited to complete when attending clinic. Cough severity and impact on quality of life were assessed using a visual analogue scale and the validated Leicester cough questionnaire. Participants were asked to list triggers of their cough and strategies or medications trialled to control cough.RESULTS:Of 164 respondents, 118 (72%) had cough, with prevalence common in all ILD subtypes. A lower forced vital capacity (FVC) was found in the cough group versus non-cough group (74.6 ± 18.7 vs 87.0 ± 15.9, P-value < 0.0001). Common reported triggers were lung irritants, exertion and doing routine daily activities. Avoidance of triggers was a common strategy to control cough. A high prevalence of non-ILD causes of cough was recorded in both groups. A variety of medications had been trialled, including anti-fibrotics, immunosuppression drugs, inhalers and proton pump inhibitors, with moderate benefit reported by 18% of participants.CONCLUSIONS:Cough is prevalent in ILD but is not adequately suppressed. Cough has a significant impact on quality of life, leading patients to adopt their own strategies to control their cough. More research is needed to understand cough mechanisms in ILD and the interplay of other potential co-pathologies.

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±12.5 (SD) years, mean FVC (%predicted) 79.1±20.4 (SD), mean DLCO (%predicted) 58.5±17.9 (SD) and nadir exertional SpO2 (%) 91±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.

Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune-related pulmonary fibrosis.