Abstract
Idiopathic pulmonary fibrosis (IPF) is defined as chronic idiopathic interstitial pneumonia in adults associated with the radiological and histopathological findings of usual interstitial pneumonia (UIP). The disease is inescapably progressive, with a median survival of 2.5-3 years if not treated adequately.
Therapy of IPF should be early and comprehensive and include oxygen therapy, physiotherapy and symptomatic pharmacological treatment, all being parts of complex palliative care, in addition to causal pharmacotherapy. Only some of the patients are suitable candidates for a lung transplant.
All the same, pharmacotherapy is the basic treatment method in patients with IPF since the time causal antifibrotic treatment has become available in 2011. Its effect in the treatment of IPF has been shown, and there are two antifibrotic preparations approved for treatment, pirfenidone and nintedanib.
Though therapy with these preparations cannot stop the fibrosing process and cannot even reverse it, it may result in marked deceleration of disease progression, resulting in extended patient survival, and/or serve as a bridge to transplantation.