Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis

Respiration ◽  
2018 ◽  
Vol 97 (4) ◽  
pp. 319-328 ◽  
Author(s):  
Motoyasu Kato ◽  
Shinichi Sasaki ◽  
Kana Kurokawa ◽  
Takahiro Nakamura ◽  
Tomoko Yamada ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Pleuroparenchymal Fibroelastosis ◽  
Lower Lung ◽  
Lung Lobes

scholarly journals Pleuroparenchymal fibroelastosis presenting with pneumothorax

2018 ◽  
Vol 6 ◽  
pp. 2050313X1876285 ◽  
Author(s):  
Yen-Yu Lin ◽  
Wen-Hu Hsu ◽  
Mei-Han Wu ◽  
Teh-Ying Chou
Keyword(s):  
Interstitial Pneumonia ◽  
Progressive Disease ◽  
Inflammatory Cell ◽  
Clinical Presentation ◽  
Usual Interstitial Pneumonia ◽  
Distinct Type ◽  
Pleuroparenchymal Fibroelastosis ◽  
Biopsy Specimens ◽  
Wedge Biopsy ◽  
Pleural Involvement

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.

scholarly journals Pulmonary Sarcoidosis with Usual Interstitial Pneumonia Distributed Predominantly in the Lower Lung Fields

2006 ◽  
Vol 45 (6) ◽  
pp. 359-362 ◽  
Author(s):  
Kouichi Nobata ◽  
Takahiko Kasai ◽  
Masaki Fujimura ◽  
Masayuki Mizuguchi ◽  
Kouichi Nishi ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Pulmonary Sarcoidosis ◽  
Lower Lung

scholarly journals Usual interstitial pneumonia in ANCA-associated vasculitis: A poor prognostic factor

2020 ◽  
Vol 106 ◽  
pp. 102338 ◽  
Author(s):  
Thibault Maillet ◽  
Tiphaine Goletto ◽  
Guillaume Beltramo ◽  
Henry Dupuy ◽  
Stéphane Jouneau ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Poor Prognostic Factor ◽  
Anca Associated Vasculitis

scholarly journals Desquamation Interstitial Pneumonia Presenting as a Solid Mass in the Lung: a Case Report.

2021 ◽  
Author(s):  
Wen Luo ◽  
Wanyu Wang ◽  
Yonghong Shi ◽  
Fang Lu ◽  
Yihua Lin
Keyword(s):  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Solid Mass ◽  
Rare Presentation ◽  
Ct Guided ◽  
Pulmonary Mass ◽  
Pet Ct ◽  
Lower Lung ◽  
Lung Ct

Abstract Background: Desquamation interstitial pneumonia (DIP) is a rare type of idiopathic interstitial pneumonia. High resolution computed tomography (HRCT) shows ground glass opacities without the peripheral reticular opacities characteristic of usual interstitial pneumonia (UIP). Here we report a case of DIP presenting as a solid mass in the lung, which is rarely described in the literature.Case presentation: A 77-year-old man presented with dry cough and dyspnea on exertion for one month was admitted to our hospital. The lung CT scan showed there was a pulmonary mass in right lower lung and the patient was scheduled for surgery. He was a sculpture artist, non-smoker and the previous medical history included only mandatory spondylitis without other connective tissue diseases. Nine days post-last CT, the FDG PET/CT demonstrated the mass in the right lower lung was significantly hypermetabolic (SUV value=8.9) but inflammatory exudate could be observed around the mass. We gave antibiotics and performed CT-guided percutaneous lung biopsy. The result showed that there were a large number of macrophages in the alveolar cavity, alveolar epithelial type II cells hyperplasia in some areas and chronic inflammation was noted in the interstitium. Then the desquamative interstitial pneumonia was diagnosed. We gave the patient glucocorticoid therapy, then the patient's symptoms disappeared gradually and the lung CT showed that the pulmonary mass was disappeared.Conclusions: Our report illustrates the rare presentation of DIP. Furthermore, for the pulmonary mass, even if appeared hypermetabolic on PET/CT, it is still necessary to make a definite pathological diagnosis before operation, so as to reduce the damage to patients.

scholarly journals Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia

2020 ◽  
pp. 00196-2020
Author(s):  
Keishi Sugino ◽  
Hirotaka Ono ◽  
Shigeru Shimizu ◽  
Takeyuki Kurosawa ◽  
Keiko Matsumoto ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Medical Records ◽  
Vital Capacity ◽  
Progressive Disease ◽  
Usual Interstitial Pneumonia ◽  
Lower Lobe ◽  
Disease Entity ◽  
Pleuroparenchymal Fibroelastosis ◽  
Antifibrotic Agents

BackgroundThere are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE), apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.AimThe aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice.Patients and MethodsA retrospective analysis was performed on the medical records of all patients at 2 interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these 2 groups.ResultsSurvival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF. Moreover, the change in FVC% predicted was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.ConclusionsThe efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.

Ground-Glass Attenuation in Usual Interstitial Pneumonia: Histologic Correlation and Serial Changes onThin-Section CT

1998 ◽  
Vol 39 (2) ◽  
pp. 313
Author(s):  
Jai Soung Park ◽  
Kyung Soo Lee ◽  
Deuk Lin Choi ◽  
Ki Jung Kim ◽  
Soo Taek Uh ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Ground Glass ◽  
Ground Glass Attenuation
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