title: An analysis of the levels of the soluble form of the endothelial protein C receptor in children with Henoch-Schönlein Purpura.
abstract: The pathogenesis of Henoch-Schönlein Purpura (HSP) has not been clearly defined. Inflammatory cytokines have been associated with HSP but there are only a few reports that have focused on coagulation. The endothelial protein C receptor (EPCR), which has anticoagulant and antiinflammatory activity, is the key component of the protein C pathway. Recent studies have implicated the soluble form of EPCR (sEPCR) in Wegener's granulomatosis, Behçet's disease, and systemic lupus erythematosus. The aim of this study was to evaluate the levels of sEPCR in HSP children. Twenty-two children with HSP and 17 healthy children were included. We found no significant differences (P > .05) between patient and control groups in the levels of von Willebrand factor and thrombomodulin. The median sEPCR values in the HSP group were lower than the control group (79 vs. 102 ng/mL, respectively) (P > .05). The mean sEPCR value in HSP patients with severe abdominal pain was lower than without (88.8 ± 54.9 vs. 108.2 ± 66.3 ng/mL, respectively) (P > .05). In addition, the mean IL-6 serum levels were significantly elevated in HSP patients during the acute stage of HSP (2.1 ± 1.7 vs. 1.5 ± 1.2 pg/mL, P = .038). We also observed a slight negative correlation between the levels of sEPCR and IL-6 (R = -.135, P > .05). To our knowledge, this was the first study to analyze sEPCR levels in HSP. Our results did not conclusively identify a direct role of sEPCR in HSP, but our findings warrant further investigations, especially in severe HSP cases characterized by gastrointestinal bleeding or renal involvement.
authors.affiliation: Department of Pediatric Nephrology and Rheumatology, Ankara University School of Medicine , Ankara , Turkey.
authors.forename: Fatma Semsa
authors.initials: FS
authors.lastname: Cayci
authors.forename: Mesiha
authors.initials: M
authors.lastname: Ekim
authors.forename: Yonca
authors.initials: Y
authors.lastname: Egin
authors.forename: Hafize
authors.initials: H
authors.lastname: Gökce
authors.forename: Fatos
authors.initials: F
authors.lastname: Yalcinkaya
authors.forename: Birsin
authors.initials: B
authors.lastname: Ozcakar
authors.forename: Nejat
authors.initials: N
authors.lastname: Akar
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D000074283:Endothelial Protein C Receptor; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000097:blood / Q000209:etiology; D006801:Humans; D011695:IgA Vasculitis / Q000097:blood* / Q000150:complications; D015850:Interleukin-6 / Q000097:blood; D007674:Kidney Diseases / Q000097:blood / Q000209:etiology; D008297:Male; D011956:Receptors, Cell Surface / Q000097:blood*
chemical_list: D015703:Antigens, CD; D000074283:Endothelial Protein C Receptor; C508600:IL6 protein, human; D015850:Interleukin-6; C478370:PROCR protein, human; D011956:Receptors, Cell Surface
keywords: Henoch–Schönlein purpura; child; interleukin-6; soluble endothelial protein C receptor; von Willebrand factor