New Therapeutic Strategies for Pulmonary Fibrosis

International Lung Researchers, led by DZL Scientist Jürgen Behr (DZL Location Munich, CPC-M), reach consensus on fundamental classifications for the treatment of Interstitial Lung Diseases. In an article published in the American Journal of Respiratory and Critical Care Medicine, experts (including Margaret Salisbury, Vanderbilt University Medical Center, Nashville/Tennessee, and Athos Wells, Brompton Hospital and Imperial College, London) have also addressed and filled several knowledge and evidence gaps.

Complex Treatment Strategies for Pulmonary Fibrosis: The Need for Multidisciplinary Approaches

In interstitial lung disease (ILD), there are numerous different disease trajectories, complicating decisions about anti-inflammatory and antifibrotic treatment strategies. Often, these decisions are based on limited scientific evidence. While it is assumed that the underlying mechanisms of inflammation and fibrosis play a key role, they are not yet fully understood. To make the best individual treatment decisions, various factors must be considered. However, which of these factors should take priority in different clinical scenarios can vary significantly. A particular problem is the lack of randomized controlled trials (RCTs) that could provide guidelines to properly assess these differences at the initial presentation of patients.

To better integrate the complex information on disease mechanisms, a multidisciplinary discussion (MDD) is likely the best approach. This aims to help identify meaningful treatment goals by individually assessing the courses of inflammation and fibrosis. In certain forms of ILD, such as organizing pneumonia with overlapping fibrosis, therapy could lead to a partial reversal of the disease. In contrast, with primary and secondary fibrotic NSIP (nonspecific interstitial pneumonia), stabilizing the disease progression should be the primary goal.

It is different for idiopathic pulmonary fibrosis (IPF), where the main objective in most cases is to slow the disease’s progression. Here, new biomarkers and other data could support decision-making. A central issue in the treatment of fibrotic ILDs such as IPF is that the focus is heavily on antifibrotic therapies, while the role of the immune system is often overlooked. This could miss important contributions to the disease’s development. Therefore, there is an urgent need to investigate the role of immune cells in the lungs of ILD patients more intensively. New technologies, such as spatially resolved single-cell RNA analysis, could help discover new pathways for future, personalized therapies. By better understanding the immunological and fibrotic mechanisms, targeted treatment methods could be developed in the future, similar to recent successes in asthma treatment.

Artificial Intelligence in Predicting Lung Function Loss: More Research Needed

The role of artificial intelligence (AI) algorithms in predicting lung function loss needs further investigation. New high-resolution imaging technologies, such as endobronchial optical coherence tomography (OCT) and confocal laser endomicroscopy, allow for the visualization of fine changes in the lungs. These procedures could help diagnose patients with unclear findings on conventional CT scans. They are capable of distinguishing between different tissue types, such as scar tissue (fibrosis) and inflammation, which is especially important for patients who cannot undergo lung biopsies. Experts see great potential in these technologies for the future diagnosis and treatment of lung diseases.

Original publication: The Role of Inflammation and Fibrosis in Interstitial Lung Disease Treatment Decisions. Behr, J., Salisbury, M.L., Walsh, S.L.F., et al. Am J Respir Crit Care Med. 2024 Aug 15;210(4):392-400.

Source: DZL