An efficacy of ninedanib in patients with idiopathic pulmonary fibrosis (two case reports)

Abstract

Idiopathic pulmonary fibrosis (IPF) is an irreversible progressive diffuse parenchymal lung disease with the median survival of 3 to 5 years. However, antifibrotic therapy can significantly reduce IPF progression rate. The natural course of the disease is difficult to predict in an individual patient. The prognosis and the response to therapy could differ in different IPF patients. Two clinical cases with modified rate of IPF progression under the treatment with nintedanib are described in the article. The late initiation of therapy with nintedanib in a patient with rapidly progressive disease did not slow down the progression rate, while a patient with a relatively favorable course of IPF demonstrated less rapid worsening in clinical and functional parameters under the treatment with nintedanib.Conclusion. The time-course of clinical and functional parameters is individually related to the disease severity and the potential therapeutic efficacy. The rapidly progressive course of IPF should be considered as a criterion for early initiation of antifibrotic therapy as this treatment could improve the prognosis. Effects of antifibrotic therapy should be assessed using a complex of parameters described the disease severity because IPF can differ in clinical course and progression rate.