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Updated: Jul 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Smoking-related interstitial lung disease: radiologic-clinical-pathologic correlation
Anil K Attili1, Ella A Kazerooni, Barry H Gross
1Department of Radiology, Division of Cardiothoracic Radiology, University of Michigan Medical Center, Ann Arbor, MI 48109-2713, USA. aattili@umich.edu
Cigarette smoking is a major cause of interstitial lung diseases (ILDs), including respiratory bronchiolitis ILD, desquamative interstitial pneumonitis, and pulmonary Langerhans cell histiocytosis. High-resolution CT aids in diagnosing these smoking-related ILDs, though features can overlap.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Cigarette smoking is a significant risk factor for interstitial lung diseases (ILDs).
- Specific smoking-related ILDs (SR-ILDs) include respiratory bronchiolitis ILD (RB-ILD), desquamative interstitial pneumonitis (DIP), and pulmonary Langerhans cell histiocytosis (PLCH).
- Smokers may also have an increased risk for idiopathic pulmonary fibrosis (IPF).
Purpose of the Study:
- To review the role of cigarette smoking in the development of various ILDs.
- To discuss the diagnostic utility of high-resolution computed tomography (HRCT) in characterizing SR-ILDs.
- To highlight the overlapping clinical, radiologic, and histologic features among SR-ILDs.
Main Methods:
- Review of current literature on smoking-related interstitial lung diseases.
- Analysis of HRCT findings in differentiating and classifying SR-ILDs.
- Discussion of the histopathologic features and diagnostic challenges.
Main Results:
- Strong evidence links smoking to RB-ILD, DIP, and PLCH.
- HRCT is sensitive for detecting and characterizing SR-ILDs, including patterns like lower lung fibrosis with upper lung emphysema.
- Significant overlap exists between RB-ILD and DIP, with distinct patterns of macrophage accumulation.
- PLCH diagnosis is supported by upper lung nodules and cysts in smokers.
Conclusions:
- SR-ILDs are a distinct group of lung diseases strongly associated with cigarette smoking.
- HRCT is crucial for identifying and classifying SR-ILDs, but definitive diagnosis requires integrating clinical, radiologic, and pathologic data.
- Accurate diagnosis of SR-ILDs, especially differentiating between entities like RB-ILD and DIP, necessitates a multidisciplinary approach.
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