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Updated: May 1, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary pathology in connective tissue disease.
Anatoly Urisman1, Kirk D Jones1
1Department of Pathology, University of California San Francisco, San Francisco, California.
This review details the lung pathology in autoimmune connective tissue disease-interstitial lung disease (CTD-ILD). It focuses on histologic patterns and differential diagnoses to aid in surgical lung biopsy interpretation for CTD-ILD.
Area of Science:
- Pulmonology
- Rheumatology
- Pathology
Background:
- Autoimmune connective tissue diseases (CTD) frequently involve the lungs, leading to interstitial lung disease (ILD).
- Accurate diagnosis of CTD-ILD is crucial for patient management.
- Surgical lung biopsy is a key diagnostic tool for CTD-ILD.
Purpose of the Study:
- To review the diverse histologic manifestations of CTD-ILD.
- To highlight characteristic patterns of disease in different CTDs.
- To discuss differential diagnoses for CTD-ILD based on lung biopsy findings.
Main Methods:
- Review of existing literature on CTD-ILD pathology.
- Focus on acquired autoimmune connective tissue diseases.
- Exclusion of vasculitides, heritable connective tissue disorders, and drug-induced conditions.
Main Results:
- Detailed description of specific histologic patterns associated with various CTDs.
- Emphasis on distinguishing features to aid in diagnosis.
- Discussion of overlapping features and potential mimics.
Conclusions:
- Histologic patterns on surgical lung biopsy are vital for diagnosing CTD-ILD.
- Understanding these patterns facilitates accurate differential diagnoses.
- This review provides a resource for pathologists and clinicians managing CTD-ILD.
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