Is Pirfenidone Effective in Systemic Sclerosis-related Interstitial Lung Disease?

In this study, researchers sought to assess the efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease (SSc-ILD).

In this double-blind, randomized, placebo-controlled, pilot study, patients with SSc-ILD and forced vital capacity (FVC) between 50% and 80% of the predicted (%pred) value were randomized to receive pirfenidone (2400 mg/day) or placebo for 6 months.

Of the 34 patients, FVC was stabilized/improved in 94.1% of the patients receiving pirfenidone and 76.5% of patients receiving placebo. The median absolute change in %pred FVC was – 0.55 (- 9 to 7%) in patients receiving pirfenidone and 1.0 (- 42 to 11.5%) in the control group.

Common adverse events included gastrointestinal disturbances and skin rash.

No significant beneficial effect of pirfenidone over placebo in improving/stabilizing FVC was found. The authors note that this study was underpowered and additional studies are needed to provide conclusive evidence.

Reference

Acharya N, Sharma SK, Mishra D, et al. Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease-a randomised controlled trial. Rheumatol Int. 2020;40(5):703–710. doi:10.1007/s00296-020-04565-w.