Biomarkers of Progressive Fibrosing Interstitial Lung Diseases

Abstract

Despite adequate therapy, interstitial lung diseases (ILD) can cause progressive scarring of lung tissue. This type of ILD is known as progressive fibrosing ILD (PF­-ILD). The challenge in diagnosing PF-­ILD lies in the lack of uniformly accepted criteria for a progressive fibrosing phenotype. Most authors use criteria based on clinical features and assessment of functional imaging and radiological findings over time. However, forced vital capacity (FVC) measurement is limited by its variability, and the follow­up lasts 1­2 years. The above diagnostic challenges prevent from prescribing early adequate therapy in patients with progressive ILD, indicting the need to search for new biomarkers of the progressive fibrosing phenotype. We review the most studied and informative biomarkers of fibrosis progression in patients with ILD.