What is Pulmonary Fibrosis?

Pulmonary fibrosis is a severe scarring lung disease which can progress leading to respiratory failure. Idiopathic Pulmonary Fibrosis (IPF) is the most common form. The cause of IPF is unknown but linked to genetic and environmental factors. It is more common in men compared to women and can be inherited.

PRECISE-PF: Understanding genetic lung fibrosis

We are interested in discovering how genetic mutations cause interstitial lung disease. In the lab we use spheroids made up of alveolar cells that have been generated from patient-derived iPSC to create lung-in-a-dish models. This study has been funded by the Health Research Board in Ireland.

Lead: Killian Hurley

AGE-FIBROSIS: Assessing the Pathogenicity of Alveolar Epithelial Telomeres in Pulmonary Fibrosis

We are focused on identifying the relationship between pulmonary fibrosis and telomeres, DNA sequences that shorten naturally as we age, using patient-derived iPSC models. In addition, we are interested in examining the effects of sex hormones on pulmonary fibrosis and telomere length. This study is funded by the Irish Research Council.

Lead: Mari Ozaki

circPF: Understanding the role of non-coding RNAs in alveolar epithelial damage pulmonary fibrosis

We aim to understand how alveolar epithelial cells contribute to the development of pulmonary fibrosis. We are particularly interested in the role which non-coding RNAs such as microRNAs and circular RNAs may play in this process. This study is supported by a Clement Archer Scholarship from the School of Pharmacy and Biomolecular Sciences, RCSI.

Lead: Chiara de Santi

REIDD: Assessing the potential of Digital Patient Empowered Monitoring for Remote Early Identification of Interstitial Lung Disease

We aim to determine if daily remote real-time spirometry monitoring can identify patients with rapidly progressive CTD-ILD allowing for early intervention, improving survival outcomes. This research is supported by the RCSI StAR MD Programme in collaboration with Beacon Hospital and the Irish Society for Rheumatology’s Patient Initiative Fund.

Lead: Wan Lin Ng

What is Cystic Fibrosis?

Cystic Fibrosis (CF) is an inherited chronic multi-organ disease that primarily affects the lung and digestive system. It is caused by a mutation in the gene that produces the cystic fibrosis conductance regulator (CFTR) protein and the greatest risk factor is a family history of the disease.

This project funded by a HRB Emerging Investigator Award will pair up with the clinical teams working on the real-word ‘RECOVER’ study which is examining the day-to-day effects of the latest treatmens for people with CF. It will create a patient-specific lab model of CF lung disease to investigate variable drug responses.

Lead: Irene Oglesby

ImproveCF: Investigate variable drug responses in cystic fibrosis