• Satoshi Okamori, Takanori Asakura, Keita Masuzawa, Hiroyuki Yasuda, Hirofumi Kamata, Makoto Ishii, and Tomoko Betsuyaku.
• Division of Pulmonary Medicine, Department of Medicine, Keio University School of Medicine, Tokyo, Japan.
• Medicine (Baltimore). 2017 Dec 1; 96 (49): e9081.

RationalThe efficacy of nintedanib, a multitarget receptor tyrosine kinase inhibitor, has been demonstrated in recent randomized controlled trials involving patients with idiopathic pulmonary fibrosis (IPF). However, accelerated disease progression after nintedanib discontinuation has never been reported.Patient ConcernsWe report 2 cases involving patients with a history of IPF who presented with respiratory deterioration at 3 weeks after the discontinuation of nintedanib therapy for IPF. Neither patient fulfilled the definition of "acute exacerbation of IPF" on unilateral computed tomography.DiagnosesAccelerated disease progression after the discontinuation of nintedanib therapy for IPF.InterventionsOne patient received steroid therapy. The other patient refused to undergo steroid therapy.OutcomesThe first patient showed that the affected lobe exhibited volume loss with traction bronchiectasis after receiving steroid therapy, and succumbed to pneumothorax after 3 months. The other patient was transferred to another hospital because of a decline in his general condition.LessonsTo our knowledge, this report is the first to document accelerated disease progression after the discontinuation of nintedanib therapy for IPF. Although the accurate mechanism remains unclear, the effects of nintedanib against vascular endothelial growth factor and platelet-derived growth factor receptor may play a role. Our findings suggest that physicians should carefully monitor patients with IPF after nintedanib discontinuation.

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