Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia that is often underdiagnosed on computed tomography scans. The disease process involves a combination of fibrosis involving the visceral pleura and fibroelastic changes within the subpleural lung parenchyma. Although definitive diagnosis is based on pathological evaluation, this is often not feasible and pattern recognition on CT as "definite PPFE" or "consistent with PPFE" is important given that sub group of patients will undergo rapid progression with clinical deterioration.