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.
Abstract
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
Footnotes
This article has an editorial commentary https://doi.org/10.1183/16000617.0183-2020
This article has supplementary material available from err.ersjournals.com
This trial is registered at Prospero with registry number CRD 42019136304
Provenance: Submitted article, peer reviewed
Conflict of interest: M.E. Hellemons has nothing to disclose.
Conflict of interest: C.C. Moor has nothing to disclose.
Conflict of interest: J. von der Thüsen has nothing to disclose.
Conflict of interest: M. Rossius has nothing to disclose.
Conflict of interest: A. Odink has nothing to disclose.
Conflict of interest: L.H. Thorgersen has nothing to disclose.
Conflict of interest: J. Verschakelen has nothing to disclose.
Conflict of interest: W. Wuyts reports grants from Roche and Boehringer-Ingelheim paid to his institution, outside the submitted work.
Conflict of interest: M.S. Wijsenbeek reports grants and other fees from Hoffman-La Roche and Boehringer-Ingelheim, and other fees from Galapagos, outside the submitted work.
Conflict of interest: E. Bendstrup reports grants, personal fees and non-financial support from Boehringer-Ingelheim and Roche, and personal fees from Novartis, outside the submitted work.
- Received December 13, 2019.
- Accepted January 29, 2020.
- Copyright ©ERS 2020.
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.