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

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  • Merel E. Hellemons, Erasmus Medical Center Cancer Institute
  • ,
  • Catharina C. Moor, Erasmus Medical Center Cancer Institute
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  • Jan von der Thüsen, Erasmus Medical Center Cancer Institute
  • ,
  • Mariska Rossius, Erasmus Medical Center Cancer Institute
  • ,
  • Arlette Odink, Erasmus Medical Center Cancer Institute
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  • Laila Haugen Thorgersen, Næstved Hospital
  • ,
  • Johny Verschakelen, University Hospital, Leuven, Belgium
  • ,
  • Wim Wuyts, University Hospital, Leuven, Belgium
  • ,
  • Marlies S. Wijsenbeek, Erasmus Medical Center Cancer Institute
  • ,
  • Elisabeth Bendstrup

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.

Original languageEnglish
Article number190181
JournalEuropean Respiratory Review
Number of pages12
Publication statusPublished - Jun 2020

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