Management of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is one of the most challenging diseases for chest physicians for a number of reasons, including the complexity of the diagnostic process, which requires close interaction with different specialists, and the almost invariably poor prognosis, with a 5-year survival of ∼20%. Moreover, until recently, IPF has lacked effective therapies. Following two decades of clinical trials, most of which have produced negative results, pirfenidone, a compound with broad antifibrotic, antiinflammatory, and antioxidant properties, and nintedanib, an orally available, small molecule tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF), and fibroblast growth factor (FGF) receptors, have proved equally effective in slowing down functional decline and disease progression with an acceptable safety profile. However, neither pirfenidone nor nintedanib is a cure for IPF;neither drug improves lung function and the disease continues to progress in most patients despite treatment. Likewise, the modalities for the follow-up of IPF patients are poorly defined. Accordingly, all decisions related to patient management need to be extensively discussed and agreed upon with the patients and their families. As a result, few respiratory disorders require ofchest physicians more interactive skills and more dedication than IPF.