Research updates in Pulmonary Fibrosis (PF) and Interstitial Lung Disease (ILD) webinar

Lung Foundation Australia, in collaboration with the Centre of Research Excellence in Pulmonary Fibrosis present the research updates in pulmonary fibrosis (PF) and interstitial lung disease (ILD) webinar. Join our panel of presenters, Prof. Tamera Corte, Prof. Jo Dickinson, Dr. Gabriella Tikellis, Dr. Lai-Ying Zhang and Dr. Caitlin Fermoyle, as this webinar provides an insight into the latest advancements in PF / ILD research and clinical trials. 

Over the past decade, Australia has seen remarkable progress in pulmonary fibrosis research, driven by the Centre of Research Excellence in Pulmonary Fibrosis. Through initiatives like the Australasian ILD Registry, biobank, PACT Clinical Trials Network, CREATE researcher training, and the Translation, Education and Support (TEDS) program, research spans discovery science to implementation. Patient-driven studies explore genetic and environmental causes, diagnosis, progression, symptom relief, and health economics. Clinical trials range from cryobiopsies to rehabilitation. The webinar explores three studies in Nerandomilast, Treprostinil and the TETON-2 study which mark significant progress in pulmonary fibrosis and idiopathic pulmonary fibrosis treatment. 

This webinar provides an insight into how artificial intelligence is being harnessed to improve diagnosis in interstitial lung disease (ILD). Researchers are applying machine learning to enhance existing tests like CT imaging, digital stethoscopes, and exhaled breath analysis, unlocking hidden data to detect subtle changes earlier. Tools such as SOFIA have shown promise in predicting progression faster and more accurately, while innovations like the electronic nose and AI-assisted lung sound analysis expand diagnostic possibilities. The ultimate goal is to automate reporting, identify biomarkers, and support clinical teams, ensuring earlier, more accurate diagnoses and better patient outcomes. 

The webinar explores inherited genetic changes that are major risk factor for interstitial lung diseases, alongside environmental exposures like smoking, pollution, and dust. Studies show siblings of pulmonary fibrosis patients often display early lung abnormalities, highlighting a genetic predisposition. Genomic analysis of families helps identify rare variants linked to ILD, with research focusing on how these changes impair lung function. Known genetic contributors include surfactant proteins, telomere biology genes, and immune regulation genes. Understanding these factors aids early diagnosis, guides treatment, and supports drug development. 

In healthy lungs, alveoli rely on epithelial cells, capillaries, fibroblasts, and immune cells working together to enable efficient gas exchange. In pulmonary fibrosis, the extracellular matrix thickens, disrupting this balance and causing pathology. Traditional bulk tissue analysis limited understanding, but new technologies like single cell and spatial transcriptomics now reveal how individual cells behave, interact, and form niches that drive scarring. These approaches have uncovered unique cell types and states in fibrosis, highlighting complex interactions among epithelial, fibroblast, and immune cells. Recent global studies, including Australian contributions, show fibrotic lungs contain distinct, varied niches compared to healthy tissue. 

Research priorities in pulmonary fibrosis guide efforts toward areas most meaningful to patients, caregivers, and stakeholders. In 2019, the first study identified top priorities including reversing scarring, improving lung function, preventing progression, and addressing symptom burden. These shaped national research agendas and funding, aligning work with patient needs. By 2024, priorities were revisited, with earlier accurate diagnosis, biomarker development, and fatigue interventions added. Advances in genetics, antifibrotics, artificial intelligence, and telehealth have reshaped the landscape, yet many questions remain. Ongoing priority setting ensures research continues to reflect patient voices, driving progress toward improved care, treatments, and quality of life.