Treatment of severe idiopathic pulmonary fibrosis—is sildenafil the next (in)stage?

Despite progress in the therapeutic management of idiopathic pulmonary fibrosis (IPF) in the last decade, prognosis remains poor (1). This is particularly true of patients with severe disease, with 3-year mortality predicted at 76.8% for patients with advanced disease (2). Therapeutic trials in IPF typically exclude such patients which can subsequently have a significant impact on drug availability in the clinical setting. There is a desperate unmet need for treatment options in this advanced disease cohort.