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Updated: Jun 8, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Desquamative interstitial pneumonia
Henry D Tazelaar1, Joanne L Wright, Andrew Churg
1Department of Laboratory Medicine and Pathology, Mayo Clinic in Arizona, Scottsdale, AZ 85050, USA. tazelaar.henry@mayo.edu
Abstract:
Desquamative interstitial pneumonia (DIP) is one of the rarest of the idiopathic interstitial pneumonias. It is characterized by the accumulation of macrophages in large numbers in the alveolar spaces associated with interstitial inflammation and/or fibrosis. The macrophages frequently contain light brown pigment, and because of their association with smoking have been called 'smoker's macrophages'. Lymphoid nodules are common, as is a sparse but distinct eosinophil infiltrate. Most cases of DIP are caused by cigarette smoking, but drugs and other inhaled agents, including marijuana smoke, can also produce the same disease. Although respiratory bronchiolitis-interstitial lung disease (RB-ILD) is a closely related process, there are prognostic reasons for continuing to separate it from DIP when possible. The proposed relationship of DIP to fibrotic non-specific interstitial pneumonia (NSIP) remains uncertain. The prognosis of DIP appears to be significantly better than that of fibrotic NSIP, so while there can be morphological overlap between the two, merging them into one disease may hide important prognostic information. Although the majority of DIP patients improve on treatment, some patients develop progressive irreversible fibrosis.
Insights
Desquamative interstitial pneumonia (DIP) is a rare lung disease characterized by macrophage accumulation. While often linked to smoking, other inhaled agents can cause DIP, which has a better prognosis than related fibrotic lung diseases.
Area of Science:
- Pulmonology
- Pathology
Background:
- Desquamative interstitial pneumonia (DIP) is a rare idiopathic interstitial pneumonia.
- It involves significant accumulation of macrophages in alveolar spaces, often containing brown pigment ('smoker's macrophages').
- Associated findings include interstitial inflammation, fibrosis, lymphoid nodules, and eosinophil infiltrates.
Purpose of the Study:
- To describe the key pathological features of Desquamative interstitial pneumonia (DIP).
- To discuss the etiology, including smoking and other inhaled agents.
- To differentiate DIP from related lung conditions like respiratory bronchiolitis-interstitial lung disease (RB-ILD) and fibrotic non-specific interstitial pneumonia (NSIP) based on prognostic factors.
Main Methods:
- Histopathological examination of lung tissue.
- Clinical correlation of findings with patient history, including smoking and exposure history.
- Comparative analysis with other interstitial lung diseases.
Main Results:
- DIP is characterized by abundant macrophages in alveoli, often pigmented.
- Cigarette smoking is the most common cause, but marijuana smoke and other inhaled agents can also induce DIP.
- DIP shows distinct features from RB-ILD, and its prognosis is generally better than fibrotic NSIP, despite potential morphological overlap.
Conclusions:
- DIP is a distinct clinicopathological entity, primarily associated with smoking.
- Distinguishing DIP from fibrotic NSIP is crucial for accurate prognosis and management.
- While most DIP patients respond to treatment, some may develop progressive fibrosis.
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