2009
DOI: 10.1111/j.1440-1843.2009.01624.x
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Determinants of initiation and progression of idiopathic pulmonary fibrosis

Abstract: IPF is a devastating disease with few therapeutic options. The precise aetiology of IPF remains elusive. However, our understanding of the pathologic processes involved in the initiation and progression of this disease is improving. Data on the mechanisms underlying IPF have been generated from epidemiologic investigations as well as cellular and molecular studies of human tissues. Although no perfect animal model of human IPF exists, pre-clinical animal studies have helped define pathways which are likely imp… Show more

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Cited by 67 publications
(58 citation statements)
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“…This disease is a major clinical problem, particularly in the elderly population. Numerous epidemiological studies have suggested a role for certain contributing factors but, to date, the initiating cause of IPF is still unknown [4]. Patients with IPF suffer from worsening dyspnoea and cough and can experience various patterns of progression during the disease course, eventually resulting in irreversible lung damage and decline of lung function [5].…”
Section: Pulmonary Fibrosismentioning
confidence: 99%
“…This disease is a major clinical problem, particularly in the elderly population. Numerous epidemiological studies have suggested a role for certain contributing factors but, to date, the initiating cause of IPF is still unknown [4]. Patients with IPF suffer from worsening dyspnoea and cough and can experience various patterns of progression during the disease course, eventually resulting in irreversible lung damage and decline of lung function [5].…”
Section: Pulmonary Fibrosismentioning
confidence: 99%
“…Transforming growth factor-␤ (TGF-␤) is widely considered to be the primary cytokine responsible for the induction of myofibroblast differentiation and has been implicated as being a critical factor in the development of pulmonary fibrosis in vivo (8,17,29). Fibroblasts treated with TGF-␤ differentiate to a myofibroblast phenotype that generates excess extracellular matrix proteins, expresses markers of myocytes, and exhibits enhanced contractile activity (4,5).…”
mentioning
confidence: 99%
“…This makes the virus an appropriate causal candidate for IPF. Outstanding improvements in molecular detection technology helped to discover several viruses associated with IPF including Hepatitis C Virus (HCV), adenovirus, Cytomegalovirus (CMV), Epstein-Barr virus (EBV), herpes simplex virus, human herpes viruses 7 and 8 and parvovirus B19 [5]. Among these, few have been studied in humans to determine the link between viruses and IPF.…”
Section: Viruses and Inflammationmentioning
confidence: 99%
“…One emerging hypothesis is that microscopic damage of alveolar epithelial cells and a dysregulated repair mechanism lead to lung fibrosis and in turn, IPF [4]. Since numerous factors have been suggested as initiators, IPF is now considered as more of a syndrome than a disease [5].…”
mentioning
confidence: 99%