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The local complement activation on vascular bed of patients with systemic sclerosis: a hypothesis-generating study
Cinzia Scambi1, Sara Ugolini1, T Sakari Jokiranta2
1Department of Medicine, University of Verona, Verona, Italy.
Plos One
|February 7, 2015
Summary
Systemic sclerosis (SSc) patients show local complement system activation in skin vessels, with lower levels of the regulator Membrane Cofactor Protein (MCP). This suggests potential for more severe disease complications in a subset of SSc patients.
Area of Science:
- Immunology
- Rheumatology
- Complement System Biology
Background:
- The complement system's role in systemic sclerosis (SSc) pathogenesis remains unclear.
- Previous research has not definitively established complement system involvement in SSc.
Purpose of the Study:
- To investigate the expression of the C5b-9 complement complex and its regulator, Membrane Cofactor Protein (MCP, CD46), in the skin vasculature of SSc patients.
- To analyze the association of MCP gene variants with SSc.
Main Methods:
- ELISA assay for plasma C5b-9 levels.
- Immunofluorescence and immunoblot analysis of C5b-9 and MCP in skin biopsies.
- Quantitative PCR for MCP gene promoter variants.
Main Results:
- Circulating C5b-9 levels were similar between SSc patients and controls.
- C5b-9 deposition was found in SSc skin biopsies, but not in controls.
- MCP expression was significantly reduced in SSc skin vessels and linked to specific MCP gene promoter variants.
Conclusions:
- This study provides the first evidence of local complement activation and abnormal MCP expression in SSc skin vasculature.
- Altered local complement activation may contribute to severe organ complications and disease progression in a subset of SSc patients.
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