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Scleroderma: from cell and molecular mechanisms to disease models
1Department of Medicine, Centre for Rheumatology, Royal Free and University College Medical School, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, UK. d.abraham@medsch.ucl.ac.uk
Trends in Immunology
|September 20, 2005
Summary
Systemic sclerosis (SSc) is an autoimmune disease where blood vessel damage and inflammation cause tissue fibrosis. Novel animal models reveal how these factors generate molecular signals driving the fibrotic response.
Area of Science:
- Immunology
- Pathology
- Rheumatology
Background:
- Scleroderma, or systemic sclerosis (SSc), is a complex autoimmune condition.
- It is characterized by pathological connective tissue remodeling, with early signs including vascular and immunological abnormalities.
- Progressive pathology suggests a link between microvascular damage, inflammation, and obliterative tissue fibrosis.
Purpose of the Study:
- To review how model systems have elucidated the relationships between microvascular damage, inflammation, and fibrosis in SSc.
- To discuss new data from novel animal disease models of SSc.
- To explain the molecular mechanisms driving the fibrogenic response in SSc.
Main Methods:
- Review of existing literature on SSc model systems.
- Analysis of data from novel animal models of SSc.
- Examination of molecular cues, soluble mediators, and mesenchymal cell behavior in SSc pathogenesis.
Main Results:
- Model systems provide insights into the interplay between vascular damage, inflammation, and fibrosis.
- Vascular damage and leukocyte accumulation generate molecular cues.
- These cues regulate soluble mediators that control mesenchymal cell behavior, contributing to persistent fibrosis.
Conclusions:
- Understanding the molecular mechanisms in animal models is crucial for SSc research.
- Aberrant behavior of mesenchymal cells, driven by molecular signals from vascular damage and inflammation, is key to SSc fibrosis.
- Dysregulated expression and differentiation of these components perpetuate the fibrogenic response in SSc.