Author:
Panahi Ladan,Udeani George,Scott Tenpas Andrew,Ofili Theresa,Marie Aguilar Elizabeth,Burchard Sarah,Ruth Ritenour Alexandra,Jacob Chennat April,Ahmed Nehal,Atphaisit Chairat,Chi Crystal,Cruz III Jesus,D. Deleon Monica,Lee Samantha,Mayo Zack,Mcbeth Mackenzie,Morales Mariel,N. Nwosu Jennifer,Palacios Kelly,M. Pena Jaycob,Vara Nitza
Abstract
Idiopathic pulmonary fibrosis (IPF) is a common interstitial lung disease (ILD) caused by environmental exposures, infections, or traumatic injuries and subsequent epithelial damage. Since IPF is a progressively fatal disease without remission, treatment is both urgent and necessary. The two medications indicated solely for treatment include the tyrosine kinase inhibitor nintedanib (Ofev®) and the anti-fibrotic agent pirfenidone (Esbriet®). This chapter discusses in detail the current treatment options for clinical management of IPF, specifically the mentioned two pharmacotherapeutic agents that decrease physiological progression and likely improve progression-free survival. The chapter also discusses the evolution of drug therapy in IPF management and the drawbacks and limitations learned throughout historical trials and observational studies.