IPF (idiopathic pulmonary fibrosis) is a condition in which scarring of the lungs occurs without a known cause. It is a progressive disease in which the scarring (fibrosis) generally gets worse over time, leading to worse respiratory symptoms.
The diagnosis of IPF is generally made in older individuals (over 60-70) who have certain patterns of scarring on their chest scans (e.g. UIP, or usual interstitial pneumonia pattern). Other conditions which cause lung scarring and environmental exposures are then ruled out, leading to a diagnosis of idiopathic (i.e. unknown) pulmonary fibrosis.
It is very important to catch this condition early, as there are medications available to slow down the progression of IPF. These antifibrotic medications (nintedanib and pirfenidone) do not reverse the scarring, but can half the yearly rate of decline.
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