The most lethal form of interstitial pneumonia of unknown cause, idiopathic pulmonary fibrosis (IPF), is associated with a specific radiological and histopathological pattern (the so-called "usual interstitial pneumonia" pattern) and has a median survival estimated to be between 3 and 5 years after diagnosis. However, evidence suggests that IPF has different clinical phenotypes, each of which has a different disease course over time. Individual patients' natural histories of IPF are currently unpredictable, though some genetic factors and circulating biomarkers have been linked to different prognoses. IPF may be asymptomatic in its early stages, resulting in a delayed diagnosis. Pirfenidone and nintedanib have been shown to change the course of the disease by slowing the decline in lung function.
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