Practical considerations in the pharmacologic treatment of idiopathic pulmonary fibrosis.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and deadly disease. The US Food and Drug Administration recently approved two medications for the treatment of IPF - pirfenidone and nintedanib. Given the limited clinical experience with these agents, a number of questions remain regarding their use. Both pirfenidone and nintedanib were demonstrated to reduce the rate of decline in forced vital capacity in independent large, double-blind, randomized controlled clinical trials.The successful implementation of both agents in clinical practice is dependent on many factors, including which patients to prescribe them in and managing patient expectations regarding efficacy and side effects. Pirfenidone is frequently associated with gastrointestinal upset, malaise, and rash. Nintedanib often causes diarrhea. Both drugs may cause elevations in liver-associated enzymes and require serial monitoring. Despite the side effects mentioned, these drugs are generally well tolerated in the long term. It should be emphasized to patients that these drugs do not represent a 'cure' for IPF and that the goal of therapy is stabilization of their disease. Currently many questions remain regarding the use of pirfenidone and nintedanib in IPF, including which drug to prescribe, the optimal patient population to treat, the duration of therapy, and how to define treatment success or failure. It also remains to be seen whether combination therapy with both agents will result in improved outcomes. Hopefully, future randomized controlled trials will address these issues.