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Updated: Oct 2, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Increased Complement Activation in Systemic Sclerosis Patients with Skin and Lung Fibrosis
Chiara Pellicano1, Marzia Miglionico1, Laura Romaggioli1
1Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy.
Complement hemolysis 50% (CH50) and C2 levels are elevated in systemic sclerosis (SSc) patients. Elevated CH50 may indicate disease severity, skin, and lung fibrosis in SSc.
Area of Science:
- Immunology
- Rheumatology
- Clinical Chemistry
Background:
- The role of the complement system in systemic sclerosis (SSc) pathogenesis remains debated.
- Investigating complement fractions may elucidate SSc phenotypic expression and disease progression.
Purpose of the Study:
- To measure complement fractions (CH50, C2, C3, C4) in SSc patients.
- To correlate complement levels with clinical disease manifestations and severity.
Main Methods:
- Assessed complement hemolysis 50% (CH50), C2, C3, and C4 levels in 85 SSc patients and 47 healthy controls (HC).
- Correlated complement levels with clinical parameters including modified Rodnan Skin Score (mRSS), disease severity scale (DSS), and diffusing capacity of carbon monoxide (DLco).
Main Results:
- SSc patients showed significantly higher CH50 and C2 levels compared to HC (p < 0.0001).
- Elevated CH50 levels were observed in diffuse cutaneous SSc (dcSSc) and in patients with interstitial lung disease (ILD).
- CH50 positively correlated with mRSS and DSS, and negatively with DLco.
Conclusions:
- Increased CH50 and C2 levels are present in SSc patients compared to healthy individuals.
- CH50 may serve as a potential biomarker for disease severity, skin fibrosis, and lung fibrosis in SSc.
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