Pleuroparenchymal fibroelastosis (PPFE) is a rare type of interstitial lung disease (ILD) that causes scarring and stiffening of the lungs, particularly in the upper areas. It is a progressive condition, and currently there are no specific treatments beyond lung transplantation.
A recent study by an international cohort of researchers (which included Prof Joseph Jacob from UCL Respiratory) examined lung tissue from 40 people with PPFE using advanced molecular techniques at the single nuclear level to understand which cell types are involved in driving the disease. They found that PPFE is not just a simple scarring process, but involves a complex mix of lung cells and immune cells working together in different parts of the lung.
A key finding was the identification of specific “scar-forming” cells in PPFE that are also seen in other forms of lung fibrosis, alongside immune cells that cluster together in organised groups. These immune structures appear to interact with scar-forming cells and may help drive ongoing inflammation and lung damage.
The study also showed that PPFE damage is not uniform, but organised into distinct layers within the lung, including areas of active scarring, immune activity, and changes in the lung lining cells.
Overall, the research helps build a clearer picture of how PPFE develops at a cellular level and shows that some mechanisms are shared with other types of lung fibrosis, while others appear unique to PPFE. This improves understanding of the disease and may help guide future research into targeted treatments.
You can read the full published article here
[Posted July 2026]