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Materials and methods 20 children (median age 10.6 years (1–16.6); 10 males) with PSV at various stages of disease activity were studied. PSV was classified as: polyarteritis nodosa (n = 10); Wegener's granulomatosis (n = 6); Kawasaki disease (n = 2); Behçet's disease (n = 1) and unclassified (n = 1). EPCs were detected using flow cytometry and defined as cells triple-positive for CD34, CD133, and VEGFR2. Growth factors were measured using ELISA in serum (VEGF) or platelet-poor plasma (Angiopoietin-1, Angiopoietin-2). Disease activity was assessed using a modified BVAS scoring system and evaluation of circulating endothelial cells (CECs) [1].

9.3 Endothelial progenitor cells and vasculogenic responses to therapy in children with primary systemic vasculitis