Pleuroparenchymal fibroelastosis: report of two cases in Brazil

• Autor(a) principal: Gomes,Paula Silva
• Data de Publicação: 2017
• Outros Autores: Shiang,Christina, Szarf,Gilberto, Coletta,Ester Nei Aparecida Martins, Pereira,Carlos Alberto de Castro
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Jornal Brasileiro de Pneumologia (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017000100072
• topic: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/etiology; Alveolitis, extrinsic allergic

ABSTRACT Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause.