Abstract
Idiopathic pulmonary fibrosis (IPF) is diffuse primarily fibrosing lung process of unknown etiology. The main clinical signs of IPF are middle and advanced age of patient, otherwise unexplainable breathelesness, clubbed fingers and crepitus on auscultation.
The main investigation for diagnosis is CT of lungs with high resolution algorythm with finding of usual interstitial pneumonia (UIP). Bronchoalveolar lavage has rather auxiliary differential diagnostic meaning.
In case of uncertainity the diagnosis should be supported by surgical lung biopsy with histopathologic finding of UIP. The new drug for treatment of IPF with causal action is pirfenidone.