The idiopathic pulmonary fibrosis (IPF) drugs nintedanib, pirfenidone, and sildenafil likely reduce mortality in patients with IPF, according to a systematic review of the literature and network meta-analysis of 22 approved or studied treatments for IPF. The investigational drug pamrevlumab may also reduce mortality.

The prognosis of IPF remains poor. The effectiveness of available drugs is limited, and their comparative efficacy is unclear. There are also novel treatments being developed that have not been compared.

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Here, a team of researchers led by Dena Zeraatkar, PhD, assessed the comparative effectiveness of 22 IPF drugs that have already been approved or are being studied in clinical trials. The researchers identified 48 studies that included 10,326 patients. 

They found that, with moderate certainty, nintedanib, pirfenidone, and sildenafil reduce mortality. They also found that nintedanib, nintedanib plus sildenafil, pirfenidone, pamrevlumab, and pentraxin reduce the decline of overall forced vital capacity with moderate certainty. Sildenafil is the only drug that reduced acute exacerbation and hospitalizations with moderate certainty.

Finally, the researchers found that corticosteroids plus azathioprine and N-acetylcysteine increased the risk of serious adverse events compared to placebo with high certainty.

The current clinical practice guidelines conditionally recommend the use of pirfenidone and nintedanib for the treatment of IPF, and they conditionally recommend against the use of sildenafil.

The results of this study support the current recommendations for nintedanib and pirfenidone but contrast with the recommendation against sildenafil, based on the results of 2 sildenafil studies since the publication of the guidelines.

“Future guidelines should consider sildenafil for IPF and further research needs to be done on promising IPF treatments such as pamrevlumab and pentraxin as phase 3 trials are completed,” the researchers wrote. 

The study was published in Thorax.


Pitre T, Mah J, Helmeczi W, et al. Medical treatments for idiopathic pulmonary fibrosis: a systematic review and network meta-analysis. Thorax. Published online February 10, 2022. doi:10.1136/thoraxjnl-2021-217976