Desquamative interstitial pneumonia: a systematic review of its features and outcomes. (24th June 2020)

• Record Type: Journal Article
• Authors: Hellemons, Merel E.; Moor, Catharina C.; von der Thüsen, Jan; Rossius, Mariska; Odink, Arlette; Thorgersen, Laila Haugen; Verschakelen, Johny; Wuyts, Wim; Wijsenbeek, Marlies S.; Bendstrup, Elisabeth
• Is Part Of: European respiratory review. Volume 29:Number 156(2020)
• Publication Date: 2020-06-24
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases; Respiratory organs -- Diseases; Respiratory organs -- Diseases -- Treatment; Electronic journals; Periodical; Periodicals; Periodicals; 616.2
• DOI: 10.1183/16000617.0181-2019
• Languages: English
• ISSNs: 0905-9180

Abstract

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