Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease (ILD) of unknown origin; its prognosis is very poor, with a mean survival of about 2.5–5 years after diagnosis. IPF is associated with a pattern of lung fibrosis termed usual interstitial pneumonia (UIP) detectable on high-resolution computed tomography (HRCT) or using lung biopsies. Scar tissue progressively replaces normal lung parenchyma leading to a progressive decline in lung function. Notably, disease progression may vary: some patients experience a very rapid decline, while others undergo a slower decline over time with periods of clinical stability and episodes of rapid respiratory deterioration defined by acute exacerbations.
Mortality following an acute exacerbation episode is estimated to be over 50%. Many therapies for the treatment of IPF have been evaluated over three decades of clinical trials and most of them have been shown to be ineffective or even showing harmful potential. Two anti-fibrotic drugs (pirfenidone and nintedanib) are now available for clinical use and considered as standard-of-care therapies. These drugs have been shown to slow down the decline of lung function in the clinical trials upon which their approval was based. However, their effects on the long-term prognosis and acute exacerbations of the disease are still unknown. None of the drugs can reverse lung fibrosis and one quarter of patients may need to discontinue antifibrotic treatment due to tolerability concerns. This, together with the emerging evidence of different phenotypes in IPF, and the scarcity of biomarkers predicting the treatment response or disease progression in individual patients renders it an area where the therapeutic need must be improved.
Overall, the aim of this Topic is to explore the molecular mechanism underlying the pathogenesis and progression of IPF in order to gain a deeper understanding of this disease and facilitate proper pharmacological interventions.
In this Research Topic, we welcome Original Research, Review and Methods articles, including but not limited to the following subjects:
• Novel preclinical models to IPF.
• Biomarkers as predictors of disease behavior.
• Potential pharmacotherapeutic targets.
• Advances in drug design based on molecular targets.
Dr. Silvia Pontis is a Senior Scientist at Chiesi Farmaceutici and Dr. Paolo Spagnolo has received consulting fees from PPM Services, Galapagos, Chiesi, Santhera Pharmaceuticals and institutional grants from PPM Services, Boehringer-Ingelheim, Roche. All other Topic Editors declare no competing interests with regards to the Research Topic subject.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease (ILD) of unknown origin; its prognosis is very poor, with a mean survival of about 2.5–5 years after diagnosis. IPF is associated with a pattern of lung fibrosis termed usual interstitial pneumonia (UIP) detectable on high-resolution computed tomography (HRCT) or using lung biopsies. Scar tissue progressively replaces normal lung parenchyma leading to a progressive decline in lung function. Notably, disease progression may vary: some patients experience a very rapid decline, while others undergo a slower decline over time with periods of clinical stability and episodes of rapid respiratory deterioration defined by acute exacerbations.
Mortality following an acute exacerbation episode is estimated to be over 50%. Many therapies for the treatment of IPF have been evaluated over three decades of clinical trials and most of them have been shown to be ineffective or even showing harmful potential. Two anti-fibrotic drugs (pirfenidone and nintedanib) are now available for clinical use and considered as standard-of-care therapies. These drugs have been shown to slow down the decline of lung function in the clinical trials upon which their approval was based. However, their effects on the long-term prognosis and acute exacerbations of the disease are still unknown. None of the drugs can reverse lung fibrosis and one quarter of patients may need to discontinue antifibrotic treatment due to tolerability concerns. This, together with the emerging evidence of different phenotypes in IPF, and the scarcity of biomarkers predicting the treatment response or disease progression in individual patients renders it an area where the therapeutic need must be improved.
Overall, the aim of this Topic is to explore the molecular mechanism underlying the pathogenesis and progression of IPF in order to gain a deeper understanding of this disease and facilitate proper pharmacological interventions.
In this Research Topic, we welcome Original Research, Review and Methods articles, including but not limited to the following subjects:
• Novel preclinical models to IPF.
• Biomarkers as predictors of disease behavior.
• Potential pharmacotherapeutic targets.
• Advances in drug design based on molecular targets.
Dr. Silvia Pontis is a Senior Scientist at Chiesi Farmaceutici and Dr. Paolo Spagnolo has received consulting fees from PPM Services, Galapagos, Chiesi, Santhera Pharmaceuticals and institutional grants from PPM Services, Boehringer-Ingelheim, Roche. All other Topic Editors declare no competing interests with regards to the Research Topic subject.
