Idiopathic Pulmonary Fibrosis and Lung Cancer

A Clinical and Pathogenesis Update

Katerina M. Antoniou; Sara Tomassetti; Eliza Tsitoura; Carlo Vancheri


Curr Opin Pulm Med. 2015;21(6):626-633. 

In This Article

Abstract and Introduction


Purpose of review 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.

Recent findings 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.

Summary 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.


Lung cancer is a frequent comorbidity in idiopathic interstitial pneumonia (IIP), with a higher incidence and reduced survival in IPF compared with other IIPs.[1] Common pathogenetic concepts in IPF and lung cancer have been recently reviewed.[2] In the current review, we focused on the epigenetic alterations involved in the pathogenesis of idiopathic pulmonary fibrosis (IPF) and cancer.