Desquamative interstitial pneumonia
Desquamative interstitial pneumonia (DIP) is an interstitial pneumonia closely related to, and thought to represent the end stage of respiratory bronchiolitis interstitial lung disease (RB-ILD) 1. It is associated with heavy smoking.
It is considered one of the rarest of idiopathic interstitial pneumonias 11. There is a recognised male predilection (M:F = 2:1).
Presentation tends to be in middle age (30-60 years of age) with progressive shortness of breath and chronic cough 4.
The vast majority of patients are heavy smokers (90%) with an average smoking history of 18 pack-years. However, other predisposing factors include:
- autoimmune/systemic disorders, e.g. rheumatoid arthritis, scleroderma 7
- infection, e.g. HIV 4
- toxin, occupational or environmental exposure, e.g. asbestos 6-7
Its name is misleading as no desquamation of alveolar epithelium is present, but rather the cells that fill the alveoli are pigment-laden macrophages. This is more pronounced than in RB-ILD.
Pulmonary function tests
Pulmonary restriction is usually absent or slight, although diffusion is impaired in most patients 9.
- may show bilateral interstitial infiltrates 8: they may have a greater predilection for the lower-lung zones and, sometimes, peripheral predominance 9
- has been classically described as one of triangular shaped regions of increased density radiating from the hilar regions to the periphery; although, this is seen in only a minority of cases 10
DIP is usually characterised by diffuse ground-glass opacities (sometimes has a granular or nodular texture), which correlate histologically with the spatially homogeneous intra-alveolar accumulation of macrophages and thickening of alveolar septa:
- bilateral and symmetric (86%) 7
- basal and peripheral (60%)
- patchy (20%)
- diffuse (20%) 4
Other frequent CT findings include spatially limited, irregular linear opacities and small cystic spaces, which are indicative of fibrotic change (50% of patients 7).
Despite differences in the CT appearance of RB-ILD and DIP, imaging findings may overlap and may be indistinguishable from each other. To improve diagnostic accuracy, lung biopsy is required in all cases of suspected RB-ILD or DIP.
Honeycombing may be seen in less than one-third of cases, and if present tends to be usually peripheral and limited in extent 9.
Treatment and prognosis
With smoking cessation and corticosteroid therapy, the prognosis is good.The overall prognosis of has been described as been significantly better than that of fibrotic NSIP 11. Nevertheless, progressive disease with eventual death can occur, notably in patients with continued cigarette smoking.
For diffuse ground glass changes on CT consider the differential for ground glass opacities.
Interstitial lung disease
interstitial lung disease
- drug-induced interstitial lung disease
- hypersensitivity pneumonitis
idiopathic interstitial pneumonia (mnemonic)
- acute interstitial pneumonia (AIP)
- cryptogenic organising pneumonia (COP)
- desquamative interstitial pneumonia (DIP)
- idiopathic nonspecific interstitial pneumonia (NSIP)
- idiopathic pleuroparenchymal fibroelastosis
- lymphoid interstitial pneumonia (LIP)
- respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
- usual interstitial pneumonia / idiopathic pulmonary fibrosis (UIP/IPF)
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- 3. Heyneman LE, Ward S, Lynch DA et-al. Respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, and desquamative interstitial pneumonia: different entities or part of the spectrum of the same disease process? AJR Am J Roentgenol. 1999;173 (6): 1617-22. AJR Am J Roentgenol (abstract) - Pubmed citation
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- 8. Ryu JH, Myers JL, Capizzi SA et-al. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005;127 (1): 178-84. doi:10.1378/chest.127.1.178 - Pubmed citation
- 9. Godbert B, Wissler MP, Vignaud JM. Desquamative interstitial pneumonia: an analytic review with an emphasis on aetiology. Eur Respir Rev. 2013;22 (128): 117-23. doi:10.1183/09059180.00005812 - Pubmed citation
- 10. King MB, Jessurun J, Hertz MI. Recurrence of desquamative interstitial pneumonia after lung transplantation. Am. J. Respir. Crit. Care Med. 1997;156 (6): 2003-5. doi:10.1164/ajrccm.156.6.9703001 - Pubmed citation
- 11. Tazelaar HD, Wright JL, Churg A. Desquamative interstitial pneumonia. Histopathology. 2011;58 (4): 509-16. doi:10.1111/j.1365-2559.2010.03649.x - Pubmed citation