Active Lungs & Breathing NIHR-supported project Heart, Stroke & Blood

PROgressive lung fibrosis and 68Ga-FAPi-PET Longitudinal evaLuation for Early disease activity Readout

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A radioactive dye that already lights up active scarring in one lung disease is now being tested in the most common and deadly form of pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) destroys lung tissue, causing progressive coughing and breathlessness. Diagnosis is slow and difficult, requiring multiple tests, and the disease's course is unpredictable—some patients deteriorate rapidly while others survive for years. Current drugs offer limited benefit, and survival rates remain poor. Doctors lack a reliable, non-invasive way to tell which patients will progress quickly and need aggressive treatment early. If this imaging technique works in IPF as it does in other interstitial lung diseases, it could give clinicians a direct readout of active disease activity in the lungs. That would allow them to identify high-risk patients sooner, tailor treatment more precisely, and avoid delays that currently cost patients precious time. The scan is already used in other conditions, so if validated here it could be adopted into NHS practice relatively quickly, changing how this devastating disease is monitored and managed.

View original technical description
Pulmonary fibrosis or Interstitial Lung Disease (ILD) encompasses a wide spectrum of progressive and irreversible disorders destruction of the lung. Clinically this causes cough and progressive breathing difficulty. The commonest of these conditions, idiopathic pulmonary fibrosis (IPF), affects over 32 000 individuals in the UK and accounts for 1 in every 100 deaths in this country each year. Despite the recent approval of two antifibrotic drugs for IPF the 5-year survival rate remains 25%, far worse than many common cancers.The diagnosis of IPF is frequently challenging requiring multiple tests including imaging, blood tests, lung function, bronchoscopy (camera test into the lungs) and sometimes a biopsy. This often leads to significant delays in diagnosis and treatment. To make matters worse following a diagnosis the clinical course of the disease is unpredictable with some patients surviving many years whilst others have an aggressive disease with rapid deterioration to death within 12 months.Recently we have seen that in another ILD if we inject a radiolabeld dye it localized in the links and is able to predict people at risk of progressive disease. We hope to explore whether this is the same in people with IPF.We hope this imaging may provide a non-invasive marker of disease activity. And that early signal changes in the lung of IPF patients may allow us to predict disease progression more accurately.

Researchers

Philip Molyneaux (Principal Investigator)

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