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Nintedanib has been shown to significantly reduce the annual rate of decline in the forced vital capacity (FVC) in patients with idiopathic pulmonary fibrosis (IPF) in previous randomized trials. A 71-year-old man developed exertional dyspnea and was diagnosed with IPF. Four months after treatment with nintedanib, high-resolution computed tomography findings revealed reduced areas of ground-glass opacity and consolidation; 13 months after treatment, the FVC showed improvement from 3.07 to 3.43 L, and the serum Krebs von den Lungen (KL)-6 concentration showed a decline to normal levels. We herein report a patient with IPF who was considered a super responder to nintedanib.

Remarkable Improvement in a Patient with Idiopathic Pulmonary Fibrosis after Treatment with Nintedanib