Pleuroparenchymal fibroelastosis: report of two cases in Brazil / Fibroelastose pleuroparenquimatosa: relato de dois casos no Brasil
J. bras. pneumol
;
43(1): 72-75, Jan.-Feb. 2017. graf
Article
in English
| LILACS
| ID: biblio-841258
ABSTRACT
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.
RESUMO
RESUMO 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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Lung Diseases, Interstitial
Type of study:
Etiology study
/
Prognostic study
Limits:
Adult
/
Aged
/
Humans
/
Male
Country/Region as subject:
South America
/
Brazil
Language:
English
Journal:
J. bras. pneumol
Journal subject:
Pulmonary Disease (Specialty)
Year:
2017
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal de São Paulo/BR
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