Fibrosing interstitial lung diseases (ILD) are the most severeforms of ILD that can result in rapid worsening of respiratory failure. Progressive lung fibrosis can develop in patients with both idiopathic pulmonary fibrosis and other ILD, including chronic hypersensitivity pneumonitis, nonspecific interstitial pneumonia, connective tissue disease associated ILDs and pulmonary sarcoidosis. Immunosuppressive therapy prevents progression of fibrosis only in a proportion of patients with fibrosing ILD and is frequently associated with adverseevents. Development of novel medications with antifibroticactivity, i.e. nintedanib that inhibits key pathways of pulmonary fibrogenesis, opened up a new perspective for treatment of these diseases.
Interstitial lung diseases, pulmonary fibrosis,antifibrotic therapy