Pleuroparenchymal Fibroelastosis: A Case Report

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is rare condition characterized by elastic fibrosis of the pleura and subpleuralparenchyma with upper lobe being predominantly affected. PPFE has been included in the Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias as part of the rare. The etiology of PPFE is unclear but associations with lung and bone marrow transplantation as well as chemotherapy have been proposed. In the present report, we describe a case of 72 years old male presented to our hospital with history of cough with sputum production and right chest pain for the last 3 months. Chest X-ray showed apical mass on the right lung. Surgical lung biopsy revealed PPFE in the upper lobes. To our knowledge, this is the first report in Saudi Arabia, there have been no published reports of diagnosed cases of PPFE. Conclusion: PPFE usually shows a progressive clinical course and carries a poor prognosis. There is no effective treatment for PPFE apart from lung transplantation, other medication can be used as supportive care and includes: corticosteroids, cyclophosphamide, azathioprine, N-acetylcysteine, azithromycin, sulfamethoxazole and trimethoprim.