Abstract
Idiopathic pulmonary fibrosis (IPF) belongs among diseases which are almost untreatable and come with a grim prognosis. To date IPF killed almost all the patients diagnosed with it, excluding those who had an opportunity to undergo lung transplantation which can significantly prolong the life expectancy of properly selected patients.
In 2011, new recommendations on diagnosis and treatment of IPF were created and subsequently accepted by most of the world's large respiratory medicine societies. According to these recommendations, HRCT imaging is to play a crucial role in diagnosing IPF.
As far as treatment is concerned, corticosteroids, immunosuppressants and their combinations are no longer recommended. Since 2011, pirfenidone, the first causal drug specifically developed for IPF treatment, targeting directly the fibroproliferative process via inhibition of transforming growth factor beta, is available.
Pirfenidon is indicated for treatment of patients with mild to moderate IPF. Other new drugs, influencing directly the pathogenic pathways in IPF, are in the phase of clinical evaluation.
All these crucial steps have transformed IPF from a fatal and untreatable diagnosis to a potentially curable disease and thus the prognosis for patients with IPF was substantially improved.