Pleuroparenchymal fibroelastosis: report of two cases in Brazil

Pleuroparenchymal fibroelastosis: report of two cases in Brazil

Alternative title Fibroelastose pleuroparenquimatosa: relato de dois casos no Brasil
Author Gomes, Paula Silva Autor UNIFESP Google Scholar
Shiang, Christina Autor UNIFESP Google Scholar
Szarf, Gilberto Autor UNIFESP Google Scholar
Coletta, Ester Nei Aparecida Martins Autor UNIFESP Google Scholar
Pereira, Carlos Alberto de Castro Autor UNIFESP Google Scholar
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.

A fibroelastose pleuroparenquimatosa (FEPP) é uma doença pulmonar rara, podendo ser idiopática ou associada a diversas condições. Pelo que sabemos, este é o primeiro relato de dois casos de FEPP no Brasil. Nosso primeiro paciente apresentava fibrose pleural e subpleural nos lobos superiores, um nódulo espiculado no lobo superior esquerdo e um padrão reticular discreto nos lobos inferiores. A biópsia pulmonar cirúrgica demonstrou FEPP nos lobos superiores, incluindo no nódulo, e pneumonia intersticial não classificada no lobo inferior esquerdo. Nosso segundo paciente tinha história de exposição a aves domésticas, indicando um risco de pneumonite de hipersensibilidade, e doença pulmonar avançada predominando em lobos superiores, com fibrose subpleural. Esse paciente foi submetido a transplante pulmonar. No espécime do explante, FEPP e granulomas foram identificados, sugerindo pneumonite de hipersensibilidade como causa associada.
Keywords Lung diseases, interstitial/diagnosis
Lung diseases, interstitial/etiology
Alveolitis, extrinsic allergic
Doenças pulmonares intersticiais/diagnóstico
Doenças pulmonares intersticiais/etiologia
Alveolite alérgica extrínseca
xmlui.dri2xhtml.METS-1.0.item-coverage Brasilia Df
Language Portuguese
English
Date 2017
Published in Jornal Brasileiro De Pneumologia. Brasilia Df, v. 43, n. 1, p. 72-75, 2017.
ISSN 1806-3713 (Sherpa/Romeo, impact factor)
Publisher Soc Brasileira Pneumologia Tisiologia
Extent 72-75
Origin http://dx.doi.org/10.1590/s1806-37562016000000045
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000395363100016
SciELO ID S1806-37132017000100072 (statistics in SciELO)
URI https://repositorio.unifesp.br/handle/11600/56393

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