Pleuroparenchymal fibroelastosis: report of two cases in Brazil
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 lob...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Pneumologia e Tisiologia
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| Series: | Jornal Brasileiro de Pneumologia |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017005001107&lng=en&tlng=en |
| _version_ | 1818352105688137728 |
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| author | Paula Silva Gomes Christina Shiang Gilberto Szarf Ester Nei Aparecida Martins Coletta Carlos Alberto de Castro Pereira |
| author_facet | Paula Silva Gomes Christina Shiang Gilberto Szarf Ester Nei Aparecida Martins Coletta Carlos Alberto de Castro Pereira |
| author_sort | Paula Silva Gomes |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-13T18:48:21Z |
| format | Article |
| id | doaj.art-cd90735057ac480ab4bf54c4c5549bb6 |
| institution | Directory Open Access Journal |
| issn | 1806-3756 |
| language | English |
| last_indexed | 2024-12-13T18:48:21Z |
| publisher | Sociedade Brasileira de Pneumologia e Tisiologia |
| record_format | Article |
| series | Jornal Brasileiro de Pneumologia |
| spelling | doaj.art-cd90735057ac480ab4bf54c4c5549bb62022-12-21T23:35:00ZengSociedade Brasileira de Pneumologia e TisiologiaJornal Brasileiro de Pneumologia1806-3756010.1590/s1806-37562016000000045S1806-37132017005001107Pleuroparenchymal fibroelastosis: report of two cases in BrazilPaula Silva GomesChristina ShiangGilberto SzarfEster Nei Aparecida Martins ColettaCarlos Alberto de Castro PereiraABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017005001107&lng=en&tlng=enDoenças pulmonares intersticiais/diagnósticoDoenças pulmonares intersticiais/etiologiaAlveolite alérgica extrínseca |
| spellingShingle | Paula Silva Gomes Christina Shiang Gilberto Szarf Ester Nei Aparecida Martins Coletta Carlos Alberto de Castro Pereira Pleuroparenchymal fibroelastosis: report of two cases in Brazil Jornal Brasileiro de Pneumologia Doenças pulmonares intersticiais/diagnóstico Doenças pulmonares intersticiais/etiologia Alveolite alérgica extrínseca |
| title | Pleuroparenchymal fibroelastosis: report of two cases in Brazil |
| title_full | Pleuroparenchymal fibroelastosis: report of two cases in Brazil |
| title_fullStr | Pleuroparenchymal fibroelastosis: report of two cases in Brazil |
| title_full_unstemmed | Pleuroparenchymal fibroelastosis: report of two cases in Brazil |
| title_short | Pleuroparenchymal fibroelastosis: report of two cases in Brazil |
| title_sort | pleuroparenchymal fibroelastosis report of two cases in brazil |
| topic | Doenças pulmonares intersticiais/diagnóstico Doenças pulmonares intersticiais/etiologia Alveolite alérgica extrínseca |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132017005001107&lng=en&tlng=en |
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