Awareness among clinicians about idiopathic Pleuroparenchymal Fibroelastosis (PPFE) is lacking and by the time patients are diagnosed with it, they were 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. Most common cause of fibrosis in other processes is collagen predominant but in PPFE fibrosis is usually caused by elastic fibers. Verhoeff van Gieson stain from lung biopsies in patients who presented 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 so that patients are diagnosed early.
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