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Updated: Jul 28, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Checkpoint Inhibitor-Associated Scleroderma and Scleroderma Mimics.
Michael Macklin1, Sudeep Yadav1, Reem Jan1
1Section of Rheumatology, Department of Medicine, University of Chicago Medical Center, Chicago, IL 60637, USA.
Immune checkpoint inhibitors (ICI) can cause systemic scleroderma (SSc) and mimics. ICI-SSc differs from primary SSc, and pre-existing SSc may flare after ICI treatment.
Area of Science:
- Oncology
- Rheumatology
- Immunology
Background:
- Immune checkpoint inhibitors (ICIs) are standard cancer therapy.
- ICI use is linked to autoimmune complications.
- Data on ICI-associated scleroderma is limited.
Purpose of the Study:
- To review literature on ICI-associated systemic scleroderma (ICI-SSc).
- To analyze differences between ICI-SSc and primary SSc.
- To investigate ICI-related scleroderma mimics.
Main Methods:
- Systematic literature review.
- Analysis of case reports and studies on ICI-SSc and mimics.
- Comparison of ICI-SSc features with primary SSc.
Main Results:
- ICI-SSc cases showed fewer vascular features and less seropositivity than primary SSc.
- Patients with pre-existing SSc may experience flares post-ICI.
- ICI-eosinophilic fasciitis cases were identified; ICI-scleromyxedema and ICI-scleroedema were not found.
Conclusions:
- ICI-SSc presents differently from primary SSc.
- Management of ICI-SSc requires further investigation.
- Collaborative, multi-institutional research is crucial for understanding and managing ICI-SSc.
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