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Bronchial Asthma and Idiopathic Pulmonary Fibrosis as Potential Targets for Hematopoietic Stem Cell Transplantation
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Júlio C. Voltarelli, Eduardo A. Donadi, José A. B. Martinez, Elcio O. Vianna, Willy Sarti
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease of unknown cause, most of the time associated with a poor prognosis.91 IPF shares a substantial number of clinical, roentgenographic and physiologic features with other idiopathic interstitial pneumonias and many studies imprecisely included different histopathological patterns under such denomination. However, it is now clear that the clinical label IPF should be reserved for patients with a specific form of fibrosing interstitial pneumonia denominated usual interstitial pneumonia (UIP).92 The cause, or causes, of IPF are presently unknown, but cigarette smoking has been identified as a potential risk factor for the disease. Several viruses have also been implicated in the pathogenesis of IFP, but no clear evidence points to a viral etiology. A familial form of the disease provides evidence for an important role of genetic mechanisms, however, the involved factors are still obscure. The IPF annual incidence has been estimated at 10 and 7 cases per 100,000 for males and females, respectively, on the basis of US registry data, while population-based studies put the prevalence between 3 and 20 cases per 100,000.93 The disease generally strikes subjects in the fifth and sixth decade of life.
Drug Targeting to the Lung: Chemical and Biochemical Considerations
Published in Anthony J. Hickey, Sandro R.P. da Rocha, Pharmaceutical Inhalation Aerosol Technology, 2019
Peter A. Crooks, Narsimha R. Penthala, Abeer M. Al-Ghananeem
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with few options for treatment (Kim et al. 2006), since the aetiology of the disease is poorly understood. It has a 5-year survival rate of 30%–50%. Recent studies have shown that accumulation of extracellular matrix plays an important role in IPF, and repeated alveolar injury causes fibroblast activation, proliferation, and differentiation myofibroblasts (Wolters et al. 2014, Blackwell et al. 2014). The myofibroblasts overgrow the alveolar lung tissue, and this results in an irreversible increase in the amounts of extracellular matrix (Parker et al. 2014). Current therapeutic interventions involve targeting matrix and matrix-processing enzymes, and inhibition of the collagen cross-linking enzyme, lysyl oxidase-like 2 is currently being investigated as a treatment option for IPF (Ahluwalia et al. 2014). The FDA approved drugs pirfenidone and nintedanib are also being utilized to treat IPF (King et al. 2014, Richeldi et al. 2014). It is known that a deficiency in the chaperone protein, FK506-binding protein 10 can attenuate collagen secretion and decrease extracellular collagen cross-linking, and this might provide a potentially specific and effective drug for treatment of IPF (Staab-Weijnitz et al. 2015).
Respiratory Pathophysiology
Published in Hyun Jung Kim, Biomimetic Microengineering, 2020
Brian F. Niemeyer, Alexander J. Kaiser, Kambez H. Benam
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease which results from deregulated wound healing and inflammation leading to lung fibrosis and scarring. Fibrosis of the lungs in turn prevents normal, healthy lung function leading to impaired breathing, gas exchange, and ultimately death due to respiratory failure. The prognosis for patients with IPF is quite poor with a median life expectancy of 3 years after diagnosis (Sundarakrishnan et al. 2018). The incidence of IPF has been estimated to be 130,000 in the United States, 300,000 in Europe, and 640,000 in East Asia although the prevalence is on the rise (Sundarakrishnan et al. 2018, Martinez et al. 2017). Although the exact cause IPF has yet to be determined, several factors associated with increased risk of disease have been identified including exposure to cigarette smoke, viral infection, altered bacterial loads and composition, microaspiration of gastric contents, as well as genetic susceptibility (Martinez et al. 2017). Over time, several animal models have been developed for the study of IPF; however, each of these animal systems is derived by artificially triggering fibrosis using various compounds, which may not accurately reflect IPF found in humans as the etiological agent is unknown (Sundarakrishnan et al. 2018, Chua, Gauldie, and Laurent 2005). Further, no current animal model is able to recapitulate all of the aspects of IPF in humans (Chua, Gauldie, and Laurent 2005). The discrepancies between IPF in humans and the current animal models simply highlights the need for complementary in vitro systems capable of replicating the disease in humans.
Idiopathic Pulmonary Fibrosis: What do we Know about the Role of Occupational and Environmental Determinants? A Systematic Literature Review and Meta-Analysis
Published in Journal of Toxicology and Environmental Health, Part B, 2022
A. Pauchet, A. Chaussavoine, JC Pairon, C. Gabillon, A. Didier, I. Baldi, Y. Esquirol
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD)(Cottin et al. 2019), usually observed in adults over the age of 50. Initially, common clinical features such as progressive cough, fatigue and dyspnea (Glass et al. 2022; van Manen et al. 2016) are reported and followed by a progressive restrictive pulmonary function impairment leading to an inexorably chronic respiratory failure and early mortality (Cottin et al. 2018; Lynch et al. 2016; Oda et al. 2018). Despite progress in understanding, the effectiveness of treatments remains moderate, predominantly aiming to slow disease progression (Bourke and Clague 2000; Canestaro et al. 2016; George et al. 2020; Glass et al. 2022; Oda et al. 2016; Russell, Ripamonti, and Vancheri 2016; Somogyi et al. 2018; Spagnolo et al. 2015; Tzouvelekis, Bonella, and Spagnolo 2015).