2015
DOI: 10.1371/journal.pone.0114856
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The Local Complement Activation on Vascular Bed of Patients with Systemic Sclerosis: A Hypothesis-Generating Study

Abstract: ObjectiveThe role of complement system in the pathogenesis of systemic sclerosis (SSc) has been debated during the last decade but an evident implication in this disease has never been found. We carried out an explorative study on SSc patients to evaluate the expression of soluble and local C5b-9 complement complex and its relation with a complement regulator, the Membrane Cofactor Protein (MCP, CD46) on skin vascular bed as target distinctive of SSc disease. We also analyzed two polymorphic variants in the co… Show more

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Cited by 25 publications
(19 citation statements)
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“…Among the upregulated DEGs, 10 DEGs belong to the family of IFN-inducible genes, 6 DEGs are complement components, and 3 DEGs are α -defensins ( Table 2 ). Induction of interferon signature genes and complement activation were reported in a number of autoimmune diseases including SSc and its complications [ 58 – 61 ]. Human α -defensins encode human neutrophil peptides (HNPs) and function as enhancer of phagocytosis by macrophages.…”
Section: Resultsmentioning
confidence: 99%
“…Among the upregulated DEGs, 10 DEGs belong to the family of IFN-inducible genes, 6 DEGs are complement components, and 3 DEGs are α -defensins ( Table 2 ). Induction of interferon signature genes and complement activation were reported in a number of autoimmune diseases including SSc and its complications [ 58 – 61 ]. Human α -defensins encode human neutrophil peptides (HNPs) and function as enhancer of phagocytosis by macrophages.…”
Section: Resultsmentioning
confidence: 99%
“…[6,7] Second, C5b-9 deposition was detected in capillaries of skin biopsies of SSc patients but not in healthy subjects. [8] Third, C4d deposition was found in renal peritubular capillaries of a subset of SSc patients with a poor renal outcome. [9] In our patient, systemic complement activation during severe SRC was unequivocally demonstrated by a constellation of findings: hypocomplementemia; circulating complement profile with increased levels of SC5b-9 and increased C3d/C3 and FBb/FB ratios; [10] vascular deposition of C5b-9 in injured glomerular capillaries and renal arterioles; and the capacity of the patient's serum obtained at admission to induce SC5b-9 deposits on cultured microvascular endothelial cells.…”
Section: Discussionmentioning
confidence: 99%
“… SRC Peritubular capillary C4d score associated with non-recovery of renal function [ 264 ]. C5b-9 deposition detected in skin biopsies of SSc patients but not in healthy subjects [ 265 ]. Case reports of eculizumab use: SRC with TMA in pregnancy; low C3 and C4, high C5b-9; no complement mutations.…”
Section: Evidence For the Role Of Complement In The Tmasmentioning
confidence: 99%