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- Open Access
Circulating endothelial cells and endothelial progenitor cells in childhood primary angiitis of the central nervous system
Pediatric Rheumatologyvolume 6, Article number: P276 (2008)
Primary angiitis of the central nervous system in children (cPACNS) is an inflammatory vasculitis that solely affects the CNS vessels in the absence of a systemic inflammatory process. Circulating endothelial cells (CECs) are increasingly described as biomarkers for tracking vascular injury . Additionally, bone marrow-derived endothelial progenitor cells (EPCs) are thought to play a pivotal role in the regeneration of damaged endothelium. We describe the relationship of CECs and EPCs to clinical and/or radiological disease progression in cPACNS.
Materials and methods
16 children, median age 7 years old (range 1.8–17); 9 males with cPACNS were studied. Two groups were identified, according to radiological and/or clinical progression, or non progression at >6 months from diagnosis. CECs were isolated from whole blood using immunomagnetic bead extraction. EPCs were detected using flow cytometry and were defined as mononuclear cells triple positive for CD34/CD133/CD144 and CD34/CD133/VEGFR2.
Median CEC count in progressive cPACNS was significantly raised to 480/ml (176–1152) compared to 36/ml (0–168) in non-progressive disease (p = 0.0007), 32/ml (0–152) in child control (p = 0.0050) and 24/ml (16–141) in patients with non inflammatory cerebrovascular pathology (p = 0.0016). CD34+CD133+CD144+ cells were significantly raised in patients with progressive disease compared to child controls (p = 0.005) and patients with non progressive disease (p = 0.03). There was a similar but non significant trend for EPCs expressing CD34/CD133/VEGFR2.
CECs can be used to track vascular injury due to cPACNS and differentiate progressive versus non-progressive cerebral vasculitis. We also demonstrated an increase in EPCs in progressive cPACNS, perhaps indicative of a compensatory reparative vasculogenic response.
Clarke LA, Shah V, Arrigoni F, Eleftheriou D, Hong Y, Halcox J, Klein NJ, Brogan PA: Quantitative detection of circulating endothelial cells in vasculitis: comparison of flow cytometry and immunomagnetic bead extraction. J Thromb Haemost. 2008, 6: 1025-1032. 10.1111/j.1538-7836.2008.02953.x.