Fibrotic lung diseases comprises of a group of almost 200 entities characterized by a heterogeneity in the extent of inflammation and/or fibrosis. Idiopathic Pulmonary Fibrosis (IPF) is the most common form of idiopathic interstitial pneumonias, which is progressive in nature and is associated with significant mortality. There is increasing evidence to demonstrate a similar progressive phenotype in a proportion of non-IPF fibrosing interstitial lung diseases and has been redefined as progressive fibrosing interstitial lung disease (PF-ILD). PF-ILDs are characterized by a rapid functional, symptomatic decline and a detrimental prognosis. Similar to IPF, a hallmark of these diseases is recurrent or persistent alveolar epithelial injury, followed by aberrant injury-repair process and associated disruption of extracellular matrix dynamics.
PF-ILDs may include idiopathic non-specific interstitial pneumonia, connective tissue disease-associated ILDs, chronic hypersensitivity pneumonitis, unclassifiable ILD, pneumoconiosis, and sarcoidosis. Management decisions for PF-ILD are challenging for clinicians due to the paucity of data on the efficacy of specific therapies. Therapies targeting inflammatory and/or immune responses are the mainstay for several of these conditions but are based on retrospective data, small observational studies, and/or expert opinion. Antifibrotic drugs, pirfenidone and nintedanib, have been developed and approved for the treatment of IPF. Based on the pathobiological and clinical similarities between PF-ILDs and IPF, these drugs have been shown to have similar benefit in other fibrosing ILDs. In-spite of these advances, there are several unknowns regarding the management of patients with PF-ILD.
With this Research Topic, we aim to:
• Summarize key clinical and pathobiological features of PF-ILD
• Enhance our understanding of factors influencing disease progression in PF-ILD and highlight novel serum and imaging biomarkers to better risk stratify this patient population
• Summarize recent advances and the pipeline of therapeutic strategies for PF-ILD
SBM delcares that they have received funding through their institution from United Therapeutics, Merck, and Promedior and royalties from Wolters Kluwer. All other Topic Editors declare no Conflict of Interest.
Fibrotic lung diseases comprises of a group of almost 200 entities characterized by a heterogeneity in the extent of inflammation and/or fibrosis. Idiopathic Pulmonary Fibrosis (IPF) is the most common form of idiopathic interstitial pneumonias, which is progressive in nature and is associated with significant mortality. There is increasing evidence to demonstrate a similar progressive phenotype in a proportion of non-IPF fibrosing interstitial lung diseases and has been redefined as progressive fibrosing interstitial lung disease (PF-ILD). PF-ILDs are characterized by a rapid functional, symptomatic decline and a detrimental prognosis. Similar to IPF, a hallmark of these diseases is recurrent or persistent alveolar epithelial injury, followed by aberrant injury-repair process and associated disruption of extracellular matrix dynamics.
PF-ILDs may include idiopathic non-specific interstitial pneumonia, connective tissue disease-associated ILDs, chronic hypersensitivity pneumonitis, unclassifiable ILD, pneumoconiosis, and sarcoidosis. Management decisions for PF-ILD are challenging for clinicians due to the paucity of data on the efficacy of specific therapies. Therapies targeting inflammatory and/or immune responses are the mainstay for several of these conditions but are based on retrospective data, small observational studies, and/or expert opinion. Antifibrotic drugs, pirfenidone and nintedanib, have been developed and approved for the treatment of IPF. Based on the pathobiological and clinical similarities between PF-ILDs and IPF, these drugs have been shown to have similar benefit in other fibrosing ILDs. In-spite of these advances, there are several unknowns regarding the management of patients with PF-ILD.
With this Research Topic, we aim to:
• Summarize key clinical and pathobiological features of PF-ILD
• Enhance our understanding of factors influencing disease progression in PF-ILD and highlight novel serum and imaging biomarkers to better risk stratify this patient population
• Summarize recent advances and the pipeline of therapeutic strategies for PF-ILD
SBM delcares that they have received funding through their institution from United Therapeutics, Merck, and Promedior and royalties from Wolters Kluwer. All other Topic Editors declare no Conflict of Interest.