Idiopathic pulmonary fibrosis: an altered fibroblast proliferation linked to cancer biology.
The fibrotic process that characterizes idiopathic pulmonary fibrosis (IPF) is commonly considered the result of a recurrent injury to the alveolar epithelium followed by an uncontrolled proliferation of fibroblasts. However, based on considerable scientific evidence, it has been recently hypothesized that IPF might be considered a neoproliferative disorder of the lung because this disease exhibits several pathogenic features similar to cancer. Indeed, epigenetic and genetic abnormalities, altered cell-to-cell communications, uncontrolled proliferation, and abnormal activation of specific signal transduction pathways are biological hallmarks that characterize the pathogenesis of IPF and cancer. IPF remains a disease marked by a survival of 3 years, and little therapeutic progress has been made in the last few years, underlining the urgent need to improve research and to change our approach to the comprehension of this disease. The concept of IPF as a cancer-like disease may be helpful in identifying new pathogenic mechanisms that can be borrowed from cancer biology, potentially leading to different and more effective therapeutic approaches. Such vision will hopefully increase the awareness of this disease among the public and the scientific community.
Regional Center for Interstitial and Rare Lung Diseases, Department of Clinical and Experimental Biomedicine, Section of Respiratory Diseases, University of Catania, Via S. Sofia 78, Catania, Italy. vancheri@unict.it
Carlo
C
Vancheri
D002450:Cell Communication; D049109:Cell Proliferation; D044127:Epigenesis, Genetic; D058750:Epithelial-Mesenchymal Transition; D005347:Fibroblasts / Q000378:metabolism*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000378:metabolism*; D009154:Mutation; D058628:Myofibroblasts / Q000378:metabolism*; D009369:Neoplasms / Q000378:metabolism*; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000378:metabolism
D016212:Transforming Growth Factor beta