Present and future perspectives in early diagnosis and monitoring for progressive fibrosing interstitial lung diseases

Progressive fibrosing interstitial lung diseases (PF-ILDs) represent a group of conditions of both known and unknown origin which continue to worsen despite standard treatments, leading to respiratory failure and early mortality. Given the potential to slow down progression by initiating antifibrotic therapies where appropriate, there is ample opportunity to implement innovative strategies for early diagnosis and monitoring with the goal of improving clinical outcomes. Early diagnosis can be facilitated by standardizing ILD multidisciplinary team (MDT) discussions, implementing machine learning algorithms for chest computed-tomography quantitative analysis and novel magnetic-resonance imaging techniques, as well as measuring blood biomarker signatures and genetic testing for telomere length and identification of deleterious mutations in telomere-related genes and other single-nucleotide polymorphisms (SNPs) linked to pulmonary fibrosis such as rs35705950 in the MUC5B promoter region. Assessing disease progression in the post COVID-19 era also led to a number of advances in home monitoring using digitally-enabled home spirometers, pulse oximeters and other wearable devices. While validation for many of these innovations is still in progress, significant changes to current clinical practice for PF-ILDs can be expected in the near future.

Automated summary

Progressive fibrosing interstitial lung diseases (PF-ILDs) are a group of diseases that worsen despite standard treatments, leading to respiratory failure and early mortality. Early diagnosis and monitoring can be improved through standardized multidisciplinary team discussions, machine learning algorithms, blood biomarkers, genetic testing, and home monitoring using wearable devices. Significant changes to clinical practice for PF-ILDs can be expected in the near future.