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Updated: Jun 28, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Smoking-related interstitial lung disease.
R Nagarjun Rao1, Lawrence R Goodman, Joseph F Tomashefski
1Department of Pathology, Medical College of Wisconsin, Milwaukee, WI 53226, USA. arao@mcw.edu
Smoking is linked to interstitial lung diseases (ILDs), including respiratory bronchiolitis, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis. These smoking-related ILDs may represent a spectrum, with biopsy crucial for diagnosis and management.
Area of Science:
- Pulmonology
- Pathology
- Epidemiology
Background:
- Tobacco smoking is a major cause of pulmonary diseases, including COPD and lung cancer.
- Interstitial lung diseases (ILDs) are increasingly recognized as being associated with smoking.
- Specific smoking-related ILDs include respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis.
Purpose of the Study:
- To explore the link between smoking and specific interstitial lung diseases.
- To investigate the clinical, radiologic, and pathologic similarities among smoking-related ILDs.
- To highlight the importance of distinguishing these entities for appropriate clinical management.
Main Methods:
- Review of epidemiologic evidence establishing the relationship between smoking and ILDs.
- Analysis of clinical, radiologic, and pathologic features of respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis.
- Emphasis on the role of surgical lung biopsy in differentiating these conditions.
Main Results:
- Smoking is strongly associated with respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis.
- These three conditions share significant clinical, radiologic, and pathologic features.
- Distinguishing between these entities can be challenging, even with histologic evaluation.
Conclusions:
- Smoking-related interstitial lung diseases may represent a spectrum of disease in susceptible individuals.
- Accurate diagnosis through histologic evaluation is critical due to differing management strategies.
- Further research, including genetic factors, is needed to fully understand these conditions.
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