PT  - JOURNAL ARTICLE
AU  - Merel E. Hellemons
AU  - Catharina C. Moor
AU  - Jan von der Thüsen
AU  - Mariska Rossius
AU  - Arlette Odink
AU  - Laila Haugen Thorgersen
AU  - Johny Verschakelen
AU  - Wim Wuyts
AU  - Marlies S. Wijsenbeek
AU  - Elisabeth Bendstrup
TI  - Desquamative interstitial pneumonia: a systematic review of its features and outcomes
AID  - 10.1183/16000617.0181-2019
DP  - 2020 Jun 30
TA  - European Respiratory Review
PG  - 190181
VI  - 29
IP  - 156
4099  - http://err.ersjournals.com/content/29/156/190181.short
4100  - http://err.ersjournals.com/content/29/156/190181.full
SO  - EUROPEAN RESPIRATORY REVIEW2020 Jun 30; 29
AB  - 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.This systematic review is the most comprehensive collection of published cases of DIP to date and yields novel information about aetiology, clinical characteristics, radiology and pathology features, treatment and outcomes. http://bit.ly/394YRPv