Desquamative interstitial pneumonia: a systematic review of its features and outcomes

Background Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. Methods A systematic lit...

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প্রধান লেখক: Merel E. Hellemons, Catharina C. Moor, Jan von der Thüsen, Mariska Rossius, Arlette Odink, Laila Haugen Thorgersen, Johny Verschakelen, Wim Wuyts, Marlies S. Wijsenbeek, Elisabeth Bendstrup
বিন্যাস: প্রবন্ধ
ভাষা:English
প্রকাশিত: European Respiratory Society 2020-06-01
মালা:European Respiratory Review
অনলাইন ব্যবহার করুন:http://err.ersjournals.com/content/29/156/190181.full
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author Merel E. Hellemons
Catharina C. Moor
Jan von der Thüsen
Mariska Rossius
Arlette Odink
Laila Haugen Thorgersen
Johny Verschakelen
Wim Wuyts
Marlies S. Wijsenbeek
Elisabeth Bendstrup
author_facet Merel E. Hellemons
Catharina C. Moor
Jan von der Thüsen
Mariska Rossius
Arlette Odink
Laila Haugen Thorgersen
Johny Verschakelen
Wim Wuyts
Marlies S. Wijsenbeek
Elisabeth Bendstrup
author_sort Merel E. Hellemons
collection DOAJ
description Background Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. Methods A systematic literature search was performed for all original cases of adult patients with histopathologically-confirmed DIP. Individual patient data were extracted and summarised. Results We included 68 individual cases and 13 case series reporting on 294 cases. Most common presenting symptoms were dyspnoea and cough. Pulmonary function showed a restrictive pattern (71%) with decreased diffusion capacity. We found a high incidence (81%) of ever smoking in patients with DIP and 22% of patients had other (occupational) exposures. Characteristic features on high-resolution computed tomography (HRCT) scan were bilateral ground-glass opacities with lower lobe predominance (92%). Treatment and duration of treatment widely varied. Initial response to treatment was generally good, but definitely not uniformly so. A significant proportion of patients died (25% of individual cases) or experienced a relapse (18% of individual cases). Conclusion DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology.
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spelling doaj.art-f3e6747ce6374ab0a19c2072fbe42a7e2022-12-21T23:57:14ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172020-06-012915610.1183/16000617.0181-20190181-2019Desquamative interstitial pneumonia: a systematic review of its features and outcomesMerel E. Hellemons0Catharina C. Moor1Jan von der Thüsen2Mariska Rossius3Arlette Odink4Laila Haugen Thorgersen5Johny Verschakelen6Wim Wuyts7Marlies S. Wijsenbeek8Elisabeth Bendstrup9 Dept of Interstitial Lung Disease, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Interstitial Lung Disease, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Pathology, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Respiratory Diseases, Nastved Hospital, Nastved, Denmark Dept of Radiology, University Hospital Leuven, Leuven, Belgium Unit of Interstitial Lung Disease, University Hospital Leuven, Leuven, Belgium Dept of Interstitial Lung Disease, Erasmus Medical Center, Rotterdam, The Netherlands Dept of Respiratory Diseases and Allergy, Center for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark Background Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). Data on clinical features, aetiology, prognosis and effect of treatment strategies are limited. We aimed to collect all published cases to better characterise DIP. Methods A systematic literature search was performed for all original cases of adult patients with histopathologically-confirmed DIP. Individual patient data were extracted and summarised. Results We included 68 individual cases and 13 case series reporting on 294 cases. Most common presenting symptoms were dyspnoea and cough. Pulmonary function showed a restrictive pattern (71%) with decreased diffusion capacity. We found a high incidence (81%) of ever smoking in patients with DIP and 22% of patients had other (occupational) exposures. Characteristic features on high-resolution computed tomography (HRCT) scan were bilateral ground-glass opacities with lower lobe predominance (92%). Treatment and duration of treatment widely varied. Initial response to treatment was generally good, but definitely not uniformly so. A significant proportion of patients died (25% of individual cases) or experienced a relapse (18% of individual cases). Conclusion DIP remains an uncommon disease, frequently but not always related to smoking or other exposures. Furthermore, DIP behaves as a progressive disease more often than generally thought, possibly associated with different underlying aetiology.http://err.ersjournals.com/content/29/156/190181.full
spellingShingle Merel E. Hellemons
Catharina C. Moor
Jan von der Thüsen
Mariska Rossius
Arlette Odink
Laila Haugen Thorgersen
Johny Verschakelen
Wim Wuyts
Marlies S. Wijsenbeek
Elisabeth Bendstrup
Desquamative interstitial pneumonia: a systematic review of its features and outcomes
European Respiratory Review
title Desquamative interstitial pneumonia: a systematic review of its features and outcomes
title_full Desquamative interstitial pneumonia: a systematic review of its features and outcomes
title_fullStr Desquamative interstitial pneumonia: a systematic review of its features and outcomes
title_full_unstemmed Desquamative interstitial pneumonia: a systematic review of its features and outcomes
title_short Desquamative interstitial pneumonia: a systematic review of its features and outcomes
title_sort desquamative interstitial pneumonia a systematic review of its features and outcomes
url http://err.ersjournals.com/content/29/156/190181.full
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