Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing interstitial disease of unknown origin, characterized by radiological and histological features consistent with usual interstitial pneumonia (UIP). It is marked by a progressive worsening of dyspnea and a decline in lung function. Both IPF and PPF are comparable because they have poor prognoses with a median survival time from diagnosis of around 2–4 years without antifibrotic therapy. This review shows the main specific characteristics and differences of epidemiology, pathophysiology, clinical and radiological features, treatment, and prognosis of IPF and PPF.
