Assessing quality of life of idiopathic pulmonary fibrosis patients: the INSTAGE study

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic disease characterised by progressive lung interstitial fibrosis of unknown cause [1]. IPF incidence increases with older age and clinical manifestations include dry cough, exertional dyspnoea and overall progressive deterioration of patient quality of life (QOL) [1]. In the past decade, new treatment options have become available to treat IPF, such as nintedanib, an intracellular tyrosine kinase inhibitor. The INPULSIS trials evaluated the efficacy and safety of nintedanib versus placebo in 1066 IPF patients with a diffusing capacity of the lung for carbon monoxide (DLCO) of 30–79% of the predicted value [2]. These trials showed that treatment with nintedanib slowed the rate of forced vital capacity (FVC) decline but no significant difference was observed in the St George s Respiratory Questionnaire (SGRQ) score [2]. The addition of sildenafil to nintedanib treatment does not improve outcomes to a greater extent than monotherapy with nintedanib, and it is not recommended for IPF subjects \u2028http://ow.ly/Gaq530o9kEg