A rare case of Pleuroparenchymal fibroelastosis in Sri Lanka

Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease which carries many challenges in the process of diagnosis. A 73 years old female was evaluated for exertional dyspnea with CXR PA evidence of bilateral upper lobe fibrosis and pleural thickening. HRCT revealed typical features suggestive of PPFE. Lung biopsy was not performed considering the risk. Though it is rare PPFE should be considered when evaluating bilateral apical fibrosis. Proper radiological evaluation is paramount important in the process of diagnostic evaluation. Performance of lung biopsy as an essential criterion should be reconsider with associated risks.