Clinical, radiological, and pathological features of idiopathic and secondary interstitial pneumonia cases with pleuroparenchymal fibroelastosis undergoing lung transplantation

2021 ◽  
Author(s):  
Naoya Ikegami ◽  
Naoki Nakajima ◽  
Akihiko Yoshizawa ◽  
Tomohiro Handa ◽  
Toyofumi Chen‐Yoshikawa ◽  
...  

2017 ◽  
Vol 43 (1) ◽  
pp. 72-75 ◽  
Author(s):  
Paula Silva Gomes ◽  
Christina Shiang ◽  
Gilberto Szarf ◽  
Ester Nei Aparecida Martins Coletta ◽  
Carlos Alberto de Castro Pereira

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.





CHEST Journal ◽  
2002 ◽  
Vol 122 (3) ◽  
pp. 779-784 ◽  
Author(s):  
Suzanne J. Timmer ◽  
Amir M. Karamzadeh ◽  
G.L. Yung ◽  
Jolene Kriett ◽  
Stewart W. Jamieson ◽  
...  


Respiration ◽  
2018 ◽  
Vol 97 (4) ◽  
pp. 319-328 ◽  
Author(s):  
Motoyasu Kato ◽  
Shinichi Sasaki ◽  
Kana Kurokawa ◽  
Takahiro Nakamura ◽  
Tomoko Yamada ◽  
...  




2016 ◽  
Vol 17 ◽  
pp. 8-11 ◽  
Author(s):  
Balamugesh Thangakunam ◽  
Barney T.J. Isaac ◽  
Devasahayam Jesudas Christopher ◽  
Deepak Burad


Author(s):  
E Kluka ◽  
J Jessurun ◽  
M Hertz ◽  
S Shumway ◽  
T Whelan


2014 ◽  
Vol 66 (4) ◽  
pp. 536-544 ◽  
Author(s):  
Yasuhide Takeuchi ◽  
Aya Miyagawa-Hayashino ◽  
Fengshi Chen ◽  
Takeshi Kubo ◽  
Tomohiro Handa ◽  
...  


2018 ◽  
Vol 6 ◽  
pp. 2050313X1876285 ◽  
Author(s):  
Yen-Yu Lin ◽  
Wen-Hu Hsu ◽  
Mei-Han Wu ◽  
Teh-Ying Chou

A 47-year-old woman presented with spontaneous right side pneumothorax. Image studies showed consolidations and reticular opacities involving the pleural and subpleural regions of bilateral lungs. Wedge biopsy specimens of right upper, middle and lower lobes showed fibrosis of the visceral pleura and subpleural area in all three lobes, more significant in the upper lobe. Elastic Van Gieson stain showed a pattern of alveolar septal elastosis with intra-alveolar fibrosis. The clinical presentation and pathological findings are compatible with pleuroparenchymal fibroelastosis, a rare and distinct type of interstitial lung disease. This entity is different from usual interstitial pneumonia by its relationship to pleura, upper lobe predominance and temporal homogeneity. It is different from non-specific interstitial pneumonia by its pleural involvement and scanty inflammatory cell infiltration. Pleuroparenchymal fibroelastosis is a slowly progressive disease; about half of the patients die in 10 years. No curative treatment is available at present time.



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