title: Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.
abstract: BACKGROUND
  Severe deficiency of ADAMTS13 activity leads to von Willebrand factor (VWF) ultralarge multimers with high affinity for platelets, causing thrombotic thrombocytopenic purpura. Other pathological conditions with moderate ADAMTS13 activity exhibit a thrombotic risk. We examined the ADAMTS13 activity in systemic lupus erythematosus (SLE) and its value as a thrombotic biomarker.
  
  
  METHODS
  ADAMTS13 activity, VWF antigen and multimeric structure, and vascular cell adhesion molecule 1 (VCAM-1) were measured in plasma samples from 50 SLE patients and 50 healthy donors. Disease activity (systemic lupus erythematosus disease activity index; SLEDAI) and organ damage (systemic lupus international collaborating clinics) scores, thrombotic events, antiphospholipid syndrome (APS) and antiphospholipid antibodies (aPLs) were registered.
  
  
  RESULTS
  SLE patients showed decreased ADAMTS13 activity and high VWF levels compared with controls (66 ± 27% vs. 101 ± 8%, P < 0.01, and 325 ± 151% vs. 81 ± 14%, P < 0.001). VCAM-1 levels were higher in SLE patients (P < 0.05). Considering three groups of SLE patients depending on ADAMTS13 activity (>60%, 60-40% and <40%), comparative analysis showed significant association between ADAMTS13 activity and SLEDAI (P < 0.05), presence of aPLs (P < 0.001), APS (P < 0.01) and thrombotic events (P < 0.01). Reduced ADAMTS13 activity together with increased VWF levels were especially notable in patients with active disease and with aPLs.
  
  
  CONCLUSIONS
  ADAMTS13 activity, in combination with other laboratory parameters, could constitute a potential prognostic biomarker of thrombotic risk in SLE.
authors.affiliation: Hemotherapy-Hemostasis Department.
authors.forename: S
authors.initials: S
authors.lastname: Martin-Rodriguez
authors.affiliation: Hemotherapy-Hemostasis Department.
authors.forename: J C
authors.initials: JC
authors.lastname: Reverter
authors.affiliation: Hemotherapy-Hemostasis Department.
authors.forename: D
authors.initials: D
authors.lastname: Tàssies
authors.affiliation: Systemic Autoimmune Diseases Department.
authors.forename: G
authors.initials: G
authors.lastname: Espinosa
authors.affiliation: Cardiology Department. Institut Clinic del Torax. Institut d Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Heras
authors.affiliation: Hemotherapy-Hemostasis Department.
authors.forename: M
authors.initials: M
authors.lastname: Pino
authors.affiliation: Hemotherapy-Hemostasis Department.
authors.forename: G
authors.initials: G
authors.lastname: Escolar
authors.affiliation: Hemotherapy-Hemostasis Department mdiaz@clinic.ub.es.
authors.forename: M
authors.initials: M
authors.lastname: Diaz-Ricart
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001792:Blood Platelets / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000201:enzymology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000201:enzymology / Q000473:pathology; D012307:Risk Factors; D012720:Severity of Illness Index; D013927:Thrombosis / Q000097:blood* / Q000201:enzymology / Q000473:pathology; D019010:Vascular Cell Adhesion Molecule-1 / Q000097:blood; D055815:Young Adult; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D017152:Antibodies, Antiphospholipid; D015415:Biomarkers; D019010:Vascular Cell Adhesion Molecule-1; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13 activity; SLEDAI; Systemic lupus erythematosus