Abstract
Idiopathic pulmonary fibrosis (IPF) is a disease with an unknown etiology mainly characterized by a progressive decline of lung function due to the scarring of the tissue deep in the lungs. The overall survival after diagnosis remains low between 3 and 5 years. IPF is a heterogeneous disease and much progress has been made in the past decade in understanding the disease mechanisms that contributed to the development of two new drugs, pirfenidone and nintedanib, which improved the therapeutic management of the disease. The understanding of the cofactors and comorbidities of IPF also contributed to improved management of the disease outcome. In the present review, we evaluate scientific evidence which indicates IPF as a risk factor for other diseases based on the complexity of molecular and cellular mechanisms involved in the disease development and of comorbidities. We conclude from the existing literature that while much progress has been made in understating the mechanisms involved in IPF development, further studies are still necessary to fully understand IPF pathogenesis which will contribute to the identification of novel therapeutic targets for IPF management as well as other diseases for which IPF is a major risk factor.
Highlights
Pulmonary fibrosis (PF) remains a devastating lung disease with a prognosis of 3 to 5 years of survival after diagnosis.e high morbidity and mortality rate is mostly due to limited successful treatment options except for lung transplants [1]
E exact cause of the mortality rate decrease is not known yet; a reduction in smoking is thought to have a role in the observed decline of the mortality rate of Idiopathic pulmonary fibrosis (IPF) in the study by Jeganathan [6]. e causes of PF are not always known; smoking; genetics; and environmental and occupational pollutants including cement, silica, dust, asbestos, and certain types of nanoparticles (NP) such as carbon and metal oxides all contribute to the development of PF and other interstitial lung diseases (ILD) [7]
A clinical study investigating whether sildenafil in combination with pirfenidone given to patients with advanced IPF and pulmonary hypertension (PAH) may provide a pharmacological benefit to improve survival rate showed no benefit [16]
Summary
Pulmonary fibrosis (PF) remains a devastating lung disease with a prognosis of 3 to 5 years of survival after diagnosis. A systematic review study by Hutchinson et al [2] on IPF incidence and mortality data from 21 countries between 1968 and 2012 determined that both the incidence and mortality rates beyond the year 2000 increased worldwide. In the USA alone, from 1979 to 2003, the age-adjusted mortality rate increased as well [4,5]. A more recent study by Jeganathan et al [6] found that between 2004 and 2017, the age-adjusted mortality rate for IPF decreased. E exact cause of the mortality rate decrease is not known yet; a reduction in smoking is thought to have a role in the observed decline of the mortality rate of IPF in the study by Jeganathan [6]. E exact cause of the mortality rate decrease is not known yet; a reduction in smoking is thought to have a role in the observed decline of the mortality rate of IPF in the study by Jeganathan [6]. e causes of PF are not always known (idiopathic, IPF); smoking; genetics; and environmental and occupational pollutants including cement, silica, dust, asbestos, and certain types of nanoparticles (NP) such as carbon and metal oxides all contribute to the development of PF and other interstitial lung diseases (ILD) [7]
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