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Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus
Online ISSN 1827-1723
Spagnolo P. 1, 2
1 Medical University Clinic, Canton Hospital Baselland, Liestal, Switzerland;
2 University of Basel, Basel, Switzerland
Idiopathic pulmonary fibrosis (IPF) is the most common and lethal form of fibrosing idiopathic interstitial pneumonia, with a mean survival time of 3-5 years from diagnosis. The disease, which primarily occurs in older adults, is limited to the lung and is associated with the histopathological and/or radiological pattern of usual interstitial pneumonia. Recent research in this condition has led to considerable insights into disease pathophysiology and definition as well as patient stratification. In turn, this has allowed the design of a number of high-quality phase 3 clinical trials, but the identification of an effective pharmacologic therapy for IPF has remained elusive. Finally, most recently, pirfenidone and nintedanib, two compounds pleiotropic in their mechanism of action, have been proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough for patients and physicians alike. Nevertheless, we have a long way still to go. In fact, neither pirfenidone nor nintedanib is a cure for IPF; neither drug improves lung function and most patients continue to progress despite treatment. As such, comprehensive care of patients with IPF, including management of concomitant conditions and physical debility as well as timely referral for lung transplantation, remains essential. Several agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.