Towards a better diagnosis of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias, and poses significant clinical challenges. IPF diagnosis is based on clear-cut computed tomography (CT) and histopathological criteria, in an appropriate clinical context. The diagnostic criteria include: 1) exclusion of known causes of interstitial lung disease (including connective tissue disease); 2) usual interstitial pneumonia pattern on high-resolution CT in patients not subjected to surgical lung biopsy; and 3) specific combinations of high-resolution CT with pathological patterns in case of surgical lung biopsy. Improved diagnosis of IPF may help physicians to reduce the delay before an accurate diagnosis is made and increase patient awareness and access to adequate information, follow-up and treatment.