Current treatments in the management of idiopathic pulmonary fibrosis: Pirfenidone and nintedanib

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease associated with significant morbidity and mortality. Historically, IPF has been managed using combination immunosuppressive therapy; however, this has been shown to be associated with increased mortality. Over the past 5 years, 2 disease-modifying agents have been licensed for use in IPF. Pirfenidone is a novel therapy with antifibrotic and anti-inflammatory properties. Evidence from a number of randomised control trials has shown that pirfenidone slows the progression of decline in forced vital capacity in IPF. Nintedanib is a tyrosine kinase inhibitor with antifibrotic properties which has also been shown to significantly reduce disease progression. As head-to-head comparison data are lacking, treatment selection is based on patient and clinician preference and tolerability of side effects. In the future, combination therapy with pirfenidone and nintedanib or with additional therapies may further improve lung function preservation.