Elisabeth Bendstrup, Ole Hilberg and Charlotte Hyldgaard
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive irreversible fibrotic lung disease of unknown cause. It occurs in older patients and is limited to the lungs. The prognosis is dismal with a median survival of 3-5 years after diagnosis. The diagnosis is based on a definite pattern of usual interstitial pneumonia on high resolution computed tomography or specific combinations of radiological and histopathological patterns. Early diagnosis and referrral is recommended as anti-fibrotic treatment with pirfenidone or nintedanib that can slow down progression has become available. All patients should be evaluated for lung transplantation.