Treatment with nintedanib in patients with pleuroparenchymal fibroelastosis

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a progressive fibrotic lung disease characterized by pleural thickening, pleural and parenchymal fibrosis predominately involving upper parts of the lungs. Survival is dismal and no drug has been shown to modify disease course. Aim and objectives Since parenchymal fibrosis is an important histological feature, we hypothesized that anti-fibrotic treatments might be effective...