Nintedanib in the treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease that occurs in older adults. The clinical course of IPF is variable and hard to predict in an individual patient. Nintedanib is a tyrosine kinase inhibitor that has recently been approved in the US and European Union for the treatment of IPF. Preclinical studies have shown that nintedanib interferes with processes active in fibrosis such as fibroblast proliferation, migration and differentiation and the secretion of extracellular matrix. The safety and efficacy of nintedanib have been investigated in the phase II TOMORROW trial and in two replicate 52-week randomized, placebo-controlled phase III trials known as the INPULSIS trials. These trials demonstrated that nintedanib slowed disease progression by reducing the annual rate of decline in forced vital capacity, with a manageable side-effect profile. In this review, we summarize key data supporting nintedanib as a treatment for patients with IPF and address key questions regarding the use of nintedanib in the clinical setting.

Keywords: disease progression; forced vital capacity; interstitial lung disease; safety; treatment outcome; tyrosine kinase.