Abstract
Idiopathic pulmonary fibrosis is characterized from pathogenic point of view by fibroproliferative healing of repeated multiple alveolar injuries and its radiologic and histopathological appearance is represented by usual interstitial pneumonia pattern. Prognosis of the disease is similar to that of untreatable cancers.
Until 2011 there was available no effective treatment for idiopathic pulmonary fibrosis and most of the patients died within 5 years from diagnosis and the mean survival was 2,5- 3 years. Introduction of antifibrotic agent, pirfenidone, into the treatment of idiopathic pulmonary fibrosis was important milestone, changing the disease to potentially treatable and the patients got chance for better and longer lives.