Idiopathic pulmonary fibrosis is a restrictive lung disease with high mortality and severe debilitation.  Progressive destruction of the gas-exchanging regions of the lungs results in symptoms of cough and dyspnoea, limited physical activity, and reduced quality of life and independence.  Abnormal wound-healing involving multiple signalling pathways with tyrosine kinase receptors has been shown to be involved in the pathogenesis of lung fibrosis; therefore, inhibition of these pathways is a potential target for therapy of pulmonary fibrosis.

In a 12-month randomized controlled trial involving 432 patients with idiopathic pulmonary fibrosis, the efficacy and safety of 4 different oral doses of the tyrosine kinase inhibitor BIBF 1120 was compared to placebo.  It was concluded that BIBF 1120, at a dose of 150 mg twice daily, was associated with a trend toward a reduction in the decline of forced vital capacity, with fewer acute exacerbations and preserved quality of life as compared to placebo.

Although this study failed, by a very small margin, to show statistically significant results, the evidence of safety and a trend towards improvement in the treatment of idiopathic pulmonary fibrosis warrants further investigation of the tyrosine kinase inhibitor BIBF 1120.

Full article: http://www.nejm.org/doi/full/10.1056/NEJMoa1103690#t=article
Summary: http://www.nejm.org/doi/full/10.1056/NEJMoa1103690#t=abstract

Title: Efficacy of a Tyrosine Kinase Inhibitor in Idiopathic Pulmonary Fibrosis
Authors: Richeldi L, Costabei U, Selman M, Kim DS, Hansell DM, Nicholson Age et al
Journal Title: The New England Journal of Medicine