Patients with idiopathic pulmonary fibrosis (IPF) on continuous antifibrotic therapy were associated with less future forced vital capacity (FVC) decline and lower odds of needing a lung transplant or death, according to a new study published in Respiratory Medicine.

Patients with IPF experience a progressive decline in lung function. Antifibrotic medications, such as pirfenidone and nintedanib, are intended to slow down this decline. Problems arise when patients with IPF on antifibrotic medications still experience declines in lung function.

Should physicians assume that the therapy has failed, or that lung function would have been worse if the patient was not on antifibrotic therapy in the first place? 

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“The aim of this study was to evaluate the real-world management and outcomes of patients with IPF on antifibrotic therapy who have declining lung function, using data from a large, national prospective registry,” Adams et al wrote.

“Our pre-specified hypothesis was that ongoing antifibrotic use (versus discontinuation) in the setting of FVC decline would be associated with less future decline and lower likelihood of a composite outcome of FVC decline, lung transplant, or death.”

Read more about IPF etiology

They performed a retrospective cohort study using data from the Canadian Registry for Pulmonary Fibrosis and enrolled patients with IPF between January 1, 2015, and September 29, 2019. The researchers recruited 165 patients with IPF that were on antifibrotic therapy and experienced a more than 10% decline in FVC over a 6-month period.

The results demonstrated that discontinuation in antifibrotic medication after FVC decline was associated with greater additional FVC decline when compared to continuous use. Discontinuation of antifibrotic therapy was also associated with higher odds of experiencing further FVC decline, lung transplant, or death.

“We were able to show the effect of ongoing antifibrotic therapy in the setting of FVC decline in a large, real world cohort of patients with IPF, expanding on previous data from post-hoc analyses of clinical trial populations,” the researchers concluded.


Adams CJ, Shapera S, Ryerson CJ, et al. Effect of continued antifibrotic therapy after forced vital capacity decline in patients with idiopathic pulmonary fibrosis; a real world multicenter cohort studyRespir Med. Published online December 26, 2021. doi:10.1016/j.rmed.2021.106722