Abstract
Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis and elastosis. Aim: To review the cases of PPFE found in our interstitial lung disease (ILD) outpatient clinic. Methods: Patients with histological criteria of PPFE were included. Results: We included 4 patients, which are characterized in table 1. In 1 case there was another concomitant ILD. The histology was obtained by computed tomography guided core needle biopsy (CNB) in 2 patients and by transbronchial cryobiopsy (CB) in the other 2. Conclusion: PPFE may present in earlier or advanced stages and coexist with other patterns of ILD; in this setting histological characterization of different patterns may be important. Thoracic CNB and CB may be alternative diagnostic methods in patients not amenable for surgical lung biopsy.
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