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Updated: Oct 25, 2025
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
20.4K
Interstitial lung disease pathology in systemic sclerosis.
Kristine E Konopka1, Jeffrey L Myers2
1Department of Pathology, University of Michigan, Michigan Medicine, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA.
Therapeutic Advances in Musculoskeletal Disease
|August 5, 2021
Summary
Systemic sclerosis frequently causes interstitial lung disease (ILD), most commonly fibrotic nonspecific interstitial pneumonia. Lung biopsies are often unnecessary for diagnosis, especially with typical high-resolution CT findings.
Area of Science:
- Pulmonology
- Rheumatology
- Pathology
Background:
- Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc), affecting about one-third of patients.
- Fibrotic nonspecific interstitial pneumonia (NSIP) is the predominant histological pattern in SSc-associated ILD (SSc-ILD).
- Usual interstitial pneumonia (UIP) and pleuroparenchymal fibroelastosis (PPFE)-like changes can also occur in SSc-ILD.
Purpose of the Study:
- To review the histological patterns of SSc-ILD.
- To discuss diagnostic approaches, including the role of lung biopsy.
- To outline other pulmonary findings in SSc patients.
Main Methods:
- Review of existing literature on SSc-ILD histology.
- Discussion of high-resolution computed tomography (HRCT) findings.
- Analysis of indications for surgical lung biopsy.
Main Results:
- Fibrotic NSIP is the most common ILD pattern in SSc.
- UIP is the second most common pattern.
- HRCT findings can obviate the need for biopsy in typical cases.
Conclusions:
- Histological patterns of SSc-ILD are well-defined, with fibrotic NSIP being most frequent.
- Lung biopsies are reserved for atypical presentations of SSc-ILD.
- Pulmonary vascular changes, aspiration, and diffuse alveolar damage are other potential findings in SSc patients.
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