Interstitial lung disease (ILD) is a heterogeneous group of pulmonary disorders, characterized by diffuse parenchymal lung infiltration. Although some fibrosing ILDs have the potential for improvement or stabilisation with treatment, a subset of patients will continue to progress despite immunosuppressive therapy and elimination of disease-promoting stimuli. Progressive fibrosing ILD (PF-ILD) is a new concept aiming to regroup distinct fibrotic ILD entities with progressive phenotype characterized by progressive decline in lung function worsening symptoms and fibrotic changes on high-resolution computed tomography (HRCT) scan of the chest, and quality of life, and early mortality.
Current routine clinical practice, which is done via analysis of clinical symptoms, pulmonary function tests and HRCT scans, enables the diagnosis of PF-ILD only at the later point in the disease process (significant lung decline over 10% of the residual lung function) wherein it is difficult to reverse. Multiple biomarkers have already been described in PF-ILD (Krebs von den Lungen-6 protein (KL-6), surfactant protein-D (SP-D), matrix metalloproteinases (MMPs)); however, none of them has been shown to be a sufficiently robust diagnostic/prognostic markers to justify its use in clinical practice.
In this Research Topic, we aim to highlight new potential biomarkers and technological tools in order to help clinicians in the evaluation and early diagnosis of PF-ILD patients.
Interstitial lung disease (ILD) is a heterogeneous group of pulmonary disorders, characterized by diffuse parenchymal lung infiltration. Although some fibrosing ILDs have the potential for improvement or stabilisation with treatment, a subset of patients will continue to progress despite immunosuppressive therapy and elimination of disease-promoting stimuli. Progressive fibrosing ILD (PF-ILD) is a new concept aiming to regroup distinct fibrotic ILD entities with progressive phenotype characterized by progressive decline in lung function worsening symptoms and fibrotic changes on high-resolution computed tomography (HRCT) scan of the chest, and quality of life, and early mortality.
Current routine clinical practice, which is done via analysis of clinical symptoms, pulmonary function tests and HRCT scans, enables the diagnosis of PF-ILD only at the later point in the disease process (significant lung decline over 10% of the residual lung function) wherein it is difficult to reverse. Multiple biomarkers have already been described in PF-ILD (Krebs von den Lungen-6 protein (KL-6), surfactant protein-D (SP-D), matrix metalloproteinases (MMPs)); however, none of them has been shown to be a sufficiently robust diagnostic/prognostic markers to justify its use in clinical practice.
In this Research Topic, we aim to highlight new potential biomarkers and technological tools in order to help clinicians in the evaluation and early diagnosis of PF-ILD patients.