Abstract
Interstitial lung disease (ILD) is a common complication that can develop during the course of systemic sclerosis (SSc). Nindetanib is an antifibrotic drug approved for the treatment of systemic sclerosis-associated interstitial lung disease. Although there is an insufficient data on the development of pneumothorax, the safety of Nintedanib treatment is also uncertain. We observed recurrent resistant pneumothorax under nintedanib treatment in a patient with systemic sclerosis-associated interstitial lung disease. Nintedanib use may increase the risk of developing refractory pneumothorax. Ssc patients who are started on nintedanib should be followed carefully for pneumothorax.