A commonly misdiagnosed rare pulmonary disease: Idiopathic pleuroparenchymal fibroelastosis

Awareness among clinicians about Idiopathic pleuroparenchymal fibroelastosis (PPFE) is lacking. By the time patients are diagnosed, they have been seen by multiple physicians and misdiagnosed multiple times. It is a rare condition that is characterized by fibrosis of the pleura and subpleural lung parenchyma, predominantly affecting the upper lobes. The most common cause of fibrosis in other processes is collagen predominant, but in PPFE fibrosis is usually caused by elastic fibers. A Verhoeff-Van Gieson stain from lung biopsies in patients who present with fibrosis in the upper pleural and parenchymal areas will help in establishing the diagnosis by demonstrating the elastic fibers. We also need to rule out the possibility of other lung parenchymal conditions like usual interstitial pneumonia, nonspecific interstitial pneumonitis, pulmonary apical cap, etc. We have presented a case report on PPFE to bring attention to clinicians and to add to the literature so that patients are diagnosed early.