Abstract
Idiopathic pulmonary fibrosis (IPF) belongs to pulmonary diseases with a grim prognosis and challenging treatment. It is characterized by progressive breathlessness and physical signs of clubbed fingers and crepitus found on auscultation at lung bases.
Radiologic and histopathologic findings of IPF correspond to those of usual interstitial pneumonia. The mean survival of IPF patients is 2.5 years.
Until recently, no efficient therapy of IPF existed. The year 2011 became a milestone in IPF treatment when the first antifibrotic drug, pirfenidone, was registered in most European countries.
In 2015, the second antifibrotic drug, nintedanib, came to Europe. Coincident with the development of treatment options in IPF is striving for early diagnosis of IPF in order to provide more and more patients with efficient treatment.
However, it must be realized that any antifibrotic treatment only slows down the disease progression, but cannot stop it. It means that the earlier we establish the diagnosis of IPF and start antifibrotic treatment, the longer survival can be offered to IPF patients.