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Updated: Oct 2, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Therapeutic Options for Systemic Sclerosis: Current and Future Perspectives in Tackling Immune-Mediated Fibrosis
Theodoros-Ioannis Papadimitriou1, Arjan van Caam1, Peter M van der Kraan1
1Department of Rheumatic Diseases, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands.
Abstract:
Systemic sclerosis (SSc) is a severe auto-immune, rheumatic disease, characterized by excessive fibrosis of the skin and visceral organs. SSc is accompanied by high morbidity and mortality rates, and unfortunately, few disease-modifying therapies are currently available. Inflammation, vasculopathy, and fibrosis are the key hallmarks of SSc pathology. In this narrative review, we examine the relationship between inflammation and fibrosis and provide an overview of the efficacy of current and novel treatment options in diminishing SSc-related fibrosis based on selected clinical trials. To do this, we first discuss inflammatory pathways of both the innate and acquired immune systems that are associated with SSc pathophysiology. Secondly, we review evidence supporting the use of first-line therapies in SSc patients. In addition, T cell-, B cell-, and cytokine-specific treatments that have been utilized in SSc are explored. Finally, the potential effectiveness of tyrosine kinase inhibitors and other novel therapeutic approaches in reducing fibrosis is highlighted.
Insights
Systemic sclerosis (SSc) is a severe autoimmune disease causing fibrosis. This review explores inflammation
Area of Science:
- Rheumatology and Immunology
- Autoimmune Diseases
- Fibrotic Disorders
Background:
- Systemic sclerosis (SSc) is a severe autoimmune rheumatic disease.
- Characterized by excessive skin and visceral organ fibrosis.
- High morbidity and mortality with limited disease-modifying therapies.
Purpose of the Study:
- To examine the relationship between inflammation and fibrosis in SSc.
- To provide an overview of current and novel treatment efficacy for SSc-related fibrosis.
- To highlight potential therapeutic approaches based on clinical trials.
Main Methods:
- Narrative review of selected clinical trials.
- Discussion of innate and acquired immune inflammatory pathways in SSc.
- Exploration of first-line therapies, cell-specific treatments, and novel approaches.
Main Results:
- Inflammation, vasculopathy, and fibrosis are key pathological hallmarks of SSc.
- Review of current therapies and their efficacy in managing SSc fibrosis.
- Highlighting novel treatments, including tyrosine kinase inhibitors, for fibrosis reduction.
Conclusions:
- Understanding the interplay between inflammation and fibrosis is crucial for SSc management.
- Current therapies offer some benefit, but novel approaches are needed.
- Emerging treatments show promise in diminishing SSc-related fibrosis.
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