Pleuroparenchymal fibroelastosis presenting with pneumothorax
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 an...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2018-03-01
|
| Series: | SAGE Open Medical Case Reports |
| Online Access: | https://doi.org/10.1177/2050313X18762853 |
| _version_ | 1819050387472121856 |
|---|---|
| author | Yen-Yu Lin Wen-Hu Hsu Mei-Han Wu Teh-Ying Chou |
| author_facet | Yen-Yu Lin Wen-Hu Hsu Mei-Han Wu Teh-Ying Chou |
| author_sort | Yen-Yu Lin |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-21T11:47:14Z |
| format | Article |
| id | doaj.art-f232bf48695c45e1a824b089bc9eedc3 |
| institution | Directory Open Access Journal |
| issn | 2050-313X |
| language | English |
| last_indexed | 2024-12-21T11:47:14Z |
| publishDate | 2018-03-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | SAGE Open Medical Case Reports |
| spelling | doaj.art-f232bf48695c45e1a824b089bc9eedc32022-12-21T19:05:09ZengSAGE PublishingSAGE Open Medical Case Reports2050-313X2018-03-01610.1177/2050313X18762853Pleuroparenchymal fibroelastosis presenting with pneumothoraxYen-Yu Lin0Wen-Hu Hsu1Mei-Han Wu2Teh-Ying Chou3Division of Molecular Pathology, Pathology and Laboratory Medicine Department, Taipei Veterans General Hospital, Taipei, TaiwanDivision of Thoracic Surgery, Surgery Department, Taipei Veterans General Hospital, Taipei, TaiwanDepartment of Radiology, Taipei Veterans General Hospital, Taipei, TaiwanInstitute of Clinical Medicine, National Yang-Ming University, Taipei, TaiwanA 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.https://doi.org/10.1177/2050313X18762853 |
| spellingShingle | Yen-Yu Lin Wen-Hu Hsu Mei-Han Wu Teh-Ying Chou Pleuroparenchymal fibroelastosis presenting with pneumothorax SAGE Open Medical Case Reports |
| title | Pleuroparenchymal fibroelastosis presenting with pneumothorax |
| title_full | Pleuroparenchymal fibroelastosis presenting with pneumothorax |
| title_fullStr | Pleuroparenchymal fibroelastosis presenting with pneumothorax |
| title_full_unstemmed | Pleuroparenchymal fibroelastosis presenting with pneumothorax |
| title_short | Pleuroparenchymal fibroelastosis presenting with pneumothorax |
| title_sort | pleuroparenchymal fibroelastosis presenting with pneumothorax |
| url | https://doi.org/10.1177/2050313X18762853 |
| work_keys_str_mv | AT yenyulin pleuroparenchymalfibroelastosispresentingwithpneumothorax AT wenhuhsu pleuroparenchymalfibroelastosispresentingwithpneumothorax AT meihanwu pleuroparenchymalfibroelastosispresentingwithpneumothorax AT tehyingchou pleuroparenchymalfibroelastosispresentingwithpneumothorax |