Idiopathic pulmonary fibrosis: do scientists focus on publishing rather than on clinical relevance?. Issue 6 (11th June 2020)

• Record Type: Journal Article
• Authors: Spek, C. Arnold; Duitman, JanWillem
• Is Part Of: European respiratory journal. Volume 55:Issue 6(2020)
• Publication Date: 2020-06-11
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.00811-2020
• Languages: English
• ISSNs: 0903-1936

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive chronic interstitial lung disorder characterised by excessive accumulation of extracellular matrix in the interstitial and alveolar spaces, resulting in scar formation and destruction of the normal pulmonary epithelium with consequent respiratory failure and eventual death [1]. Around 3 million people are affected by IPF, and the prognosis of these patients is devastating, with a median survival after diagnosis of approximately 3 years [2–4]. Despite the US Food and Drug Administartion approval of pirfenidone [5] and nintedanib [6] for the treatment of IPF in 2014, pharmacological treatment options for IPF remain limited. Although both drugs reduce the decline of lung function in IPF patients, they have serious side-effects, show no benefit on quality of life and do not stop nor reverse the disease [7–11]. Additional therapies to improve the prognosis and quality of life of IPF patients are thus urgently awaited [12]. Advocating the use of therapeutic treatment strategies in preclinical IPF research remains challenging https://bit.ly/39Ob77c