Increased Complement Activation in Systemic Sclerosis Patients with Skin and Lung Fibrosis.

Autor:	Pellicano C; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy., Miglionico M; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy., Romaggioli L; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy., Colalillo A; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy., Vantaggio L; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy., Napodano C; Synlab Data Medica, 35133 Padova, Italy., Callà C; Dipartimento di Scienze di Laboratorio e Infettivologiche, Fondazione Policlinico Universitario 'A. Gemelli' IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy., Gulli F; Laboratorio di Patologia Clinica, Ospedale Madre Giuseppina Vannini, 00177 Rome, Italy., Marino M; Dipartimento di Medicina e Chirurgia Traslazionale, Sezione di Patologia Generale, Fondazione Policlinico Universitario 'A. Gemelli' IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy., Basile U; Dipartimento di Scienze di Laboratorio e Infettivologiche, Fondazione Policlinico Universitario 'A. Gemelli' IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy., Rosato E; Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy.
Jazyk:	angličtina
Zdroj:	Journal of personalized medicine [J Pers Med] 2022 Feb 15; Vol. 12 (2). Date of Electronic Publication: 2022 Feb 15.
DOI:	10.3390/jpm12020284
Abstrakt:	Introduction: The involvement of complement system in the phenotypic expression of systemic sclerosis (SSc) is a debated topic. We aimed to assay complement fractions in SSc patients and to correlate their levels with the clinical course of disease. Key Points: 1. CH50 is increased in SSc patients compared to HC; 2. Serum C2 levels are increased in SSc patients compared to HC; 3. CH50 may represent a biomarker of skin and lung fibrosis severity in SSc patients. Method: Complement hemolysis 50% (CH50), C2, C3 and C4 levels have been assessed in 85 SSc patients and 47 healthy controls (HC). Results: SSc patients displayed a statistically significant higher value of CH50 [76.3 U/mL (IQR 65.8-89.4 U/mL) vs. 29.6 U/mL (IQR 24.7-34 U/mL); p < 0.0001] and of C2 [26.1 mg/L (IQR 24.1-32.1 mg/L) vs. 22.7 mg/L (IQR 20.6-24.4 mg/L); p < 0.0001] if compared to HC. Patients with diffuse cutaneous SSc (dcSSc) had higher levels of CH50 than patients with limited cutaneous SSc (lcSSc) [83.6 U/mL (IQR 72.3-102.7 U/mL) vs. 71.3 U/mL (IQR 63.7-83.6 U/mL); p = 0.003]. SSc patients with interstitial lung disease (ILD) had higher CH50 levels if compared to SSc patients without ILD [79.6 U/mL (IQR 68.3-97.4 U/mL) vs. 69.7 U/mL (54.6-85.7 U/mL); p = 0.042]. A positive linear correlation existed between CH50 and the modified Rodnan Skin Score (mRSS) (r = 0.285, p = 0.008) and disease severity scale (DSS) (r = 0.285, p = 0.005); a negative linear correlation was demonstrated between CH50 and the diffusing capacity of carbon monoxide (DLco) (r = -0.252, p = 0.012). In multiple linear regression analysis, only DSS was significant ( p = 0.01, beta coefficient 2.446). Conclusions: Our results show an increment of CH50 and serum C2 levels in SSc patients in comparison to HC; we retain that CH50 may represent a biomarker of disease severity and of skin and lung fibrosis in these patients.
Databáze:	MEDLINE
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