title: Idiopathic pulmonary fibrosis and lung cancer: a clinical and pathogenesis update.
abstract: OBJECTIVE
  About one out of 10 patients with idiopathic pulmonary fibrosis (IPF) develop lung cancer. This review provides an epidemiology and clinical update of the association of these two lethal diseases. In addition, we focus on the emerging overlapping epigenetic mechanisms in both diseases.
  
  
  RESULTS
  In a vast majority of cases, lung cancer is diagnosed during the clinical and radiological follow-up for the fibrosis. The risk of development of lung cancer in IPF is higher for older male smokers and there is a significantly higher prevalence of lung cancer in the combined IPF and emphysema syndrome compared with fibrosis only. The association of two lethal diseases, such as IPF and lung cancer, carries a very poor outcome and the correct treatment strategy, particularly for advanced forms of lung cancer, is still unclear.
  
  
  CONCLUSIONS
  The two novel drugs approved for IPF, pirfenidone and nintedanib, open a new scenario in which treated patients with fibrosis will live longer, and possibly have a lower incidence of lung cancer. However, prospective studies are urgently needed to definitively clarify the role of lung cancer treatment in the management of IPF patients. Furthermore, common epigenetic alterations may represent a promising target for therapeutic approaches in the near future.
authors.affiliation: aDepartment of Thoracic Medicine, University Hospital of Heraklion bLaboratory of Molecular and Cellular Pneumonology, Medical School, University of Crete, Crete, Greece cDepartment of Diseases of the Thorax, Pulmonary Unit, GB Morgagni Hospital, Forlì dRegional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Experimental Medicine, University Hospital Policlinico-Vittorio Emanuele, Catania, Italy.
authors.forename: Katerina M
authors.initials: KM
authors.lastname: Antoniou
authors.forename: Sara
authors.initials: S
authors.lastname: Tomassetti
authors.forename: Eliza
authors.initials: E
authors.lastname: Tsitoura
authors.forename: Carlo
authors.initials: C
authors.lastname: Vancheri
mesh_terms: D000818:Animals; D044127:Epigenesis, Genetic; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000453:epidemiology / Q000628:therapy; D015994:Incidence; D008175:Lung Neoplasms / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D015995:Prevalence; D012307:Risk Factors