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Updated: Sep 1, 2025
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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A broad look into the future of systemic sclerosis
Gabriela Riemekasten1, Jörg H W Distler2
1Clinic for Rheumatology and Clinical Immunology, University Clinic Schleswig-Holstein and University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.
Therapeutic Advances in Musculoskeletal Disease
|August 15, 2022
Summary
Systemic sclerosis (SSc) involves inflammation, vasculopathy, and fibrosis. Emerging research highlights environmental factors, regulatory autoantibodies, extracellular vesicles, and fibroblast plasticity in SSc pathogenesis.
Area of Science:
- Immunology
- Rheumatology
- Cell Biology
Background:
- Systemic sclerosis (SSc) is characterized by inflammation, vasculopathy, and fibrosis.
- Understanding SSc pathogenesis involves environmental factors, autoantibodies, and cellular mechanisms.
Purpose of the Study:
- To review emerging fields in SSc pathogenesis.
- To discuss regulatory autoantibodies, extracellular vesicles, and fibroblast plasticity.
- To highlight nuclear receptors and epigenetic modifications in SSc.
Main Methods:
- Review of current literature and expert insights.
- Focus on specific autoantibodies like anti-AT1R and anti-ETAR.
- Analysis of fibroblast phenotype, function, and signaling pathways.
Main Results:
- Environmental factors initiate SSc pathogenesis.
- Dysregulated autoantibodies (e.g., anti-AT1R, anti-ETAR) play a role.
- Fibroblast activation, nuclear receptors, and epigenetic changes drive fibrosis.
Conclusions:
- Novel insights into SSc pathogenesis are emerging.
- Fibroblast plasticity and epigenetic modifications are key to fibrosis.
- Targeting signaling pathways offers therapeutic potential for SSc.
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