Spontaneous pneumothorax during nintedanib therapy in patients with systemic sclerosis‐associated interstitial lung disease

Abstract

Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc). Nintedanib, an antifibrotic drug, has recently been approved for treating SSc-ILD. Although there have been no reports suggesting the development of pneumothorax with nintedanib use, its safety in patients with impaired lung function is unclear. We observed the development of refractory spontaneous pneumothorax during nintedanib therapy in two patients with SSc-ILD and impaired lung function. Nintedanib use for SSc-ILD, an extensive disease, may therefore increase the risk of pneumothorax. In addition, pneumothorax is more likely to be refractory in these cases; initiation of nintedanib treatment and follow-up should be considered carefully.

Systemic sclerosis related interstitial lung disease and nintedanib

“…Data in the literatüre on the use of nintedanib in severe SSc ILD is insufficient. Sumi et al (18) reported two patients with severe SSc-ILD, who developed spontaneous pneumothorax during nintedanib therapy. In a post-marketing survey in Japan, the frequency of pneumothorax in patients with IPF treated with nintedanib was low at 0.33% (17).…”

“…In a post-marketing survey in Japan, the frequency of pneumothorax in patients with IPF treated with nintedanib was low at 0.33% (17). Sumi et al (18) explained this situation as; fibrosis increases the fragility of the lungs, nintedanib, on the other hand, may increase fragility even more due to its anti-VEGF effect.…”