IDIOPATHIC PLEUROPARENCHYMAL FIBROELASTOSIS, AN UNKNOWN ENTITY: A CASE REPORT

Abstract

TYPE: Late Breaking Abstract TOPIC: Lung Pathology INTRODUCTION: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is rare condition characterized by fibrosis involving pleura and subpleural lung parenchyma, predominantly in the upper lobes. PPFE is specific clinical-pathological entity of idiopathic interstitial pneumonias (IIPs). CASE PRESENTATION: A 78-years-old male, former smoker, presented to our attention for worsening dyspnea, with a chest CT scan showing bilateral pleural thickening, multiple solid pulmonary nodules, most in the right upper lobe (10 mm), and bronchiectasis. In his past history occupational exposure to asbestos, COPD, chronic respiratory failure. He performed PET/CT showed multiple consolidations with 18F-FDG uptake in RUL, RLL and bilateral hilar lymph nodes. We performed a CT-guided percutaneous lung biopsy at RUL with histological report of fibroelastosis. DISCUSSION: Little is knowns about PPFE etiology, and most cases are considered idiopathic, although some cases are familiar, others have been reported in association with a previous bone marrow transplant or secondary to occupational exposure. Symptoms are similar to those of other interstitial pneumonias, but imaging and pathological findings have typical features of PPFE. The differential diagnoses include IPF, chronic hypersensitivity pneumonitis, pulmonary sarcoidosis, pneumoconiosis, connective tissue disease-related ILD, and pulmonary infections, although PPFE has also been reported in association with other ILD patterns, including IPF predominantly at lower lobes (pattern UIP). Few cases of PPFE presenting with positive uptake at PET/CT have been reported in literature. CONCLUSIONS: PPFE is still unknown entity, but it deserves to be suspected and early diagnosed since it is not very rare as previously believed and some patients has very poor outcome due to rapid clinical deterioration. DISCLOSURE: No significant relationships. KEYWORD: Fibroelastosis