title: Clinical course of high-risk patients diagnosed with antiphospholipid syndrome.
abstract: BACKGROUND
  The characteristics and the clinical course of antiphospholipid syndrome (APS) in high-risk patients that are positive for all three recommended tests that detect the presence of antiphospholipid (aPL) antibodies have not been described.
  
  
  METHODS
  This retrospective analysis of prospectively collected data examined patients referred to Italian Thrombosis Centers that were diagnosed with definite APS and tested positive for aPL [lupus anticoagulant (LA), anti-cardiolipin (aCL), and anti-beta2-glycoprotein I (beta2GPI) antibodies]. Laboratory data were confirmed in a central reference laboratory.
  
  
  RESULTS
  One hundred and sixty patients were enrolled in this cohort study. The qualifying events at diagnosis were venous thromboembolism (76 cases; 47.5%), arterial thromboembolism (69 cases; 43.1%) and pregnancy morbidity (11 cases; 9.7%). The remaining four patients (2.5%) suffered from catastrophic APS. The cumulative incidence of thromboembolic events in the follow-up period was 12.2% (95% CI, 9.6-14.8) after 1 year, 26.1% (95% CI, 22.3-29.9) after 5 years and 44.2% (95% CI, 38.6-49.8) after 10 years. This was significantly higher in those patients not taking oral anticoagulants as compared with those on treatment (HR=2.4 95% CI 1.3-4.1; P<0.003). Major bleeding associated with oral anticoagulant therapy was low (0.8% patient/years). Ten patients died (seven were cardiovascular deaths).
  
  
  CONCLUSIONS
  Patients with APS and triple positivity for aPL are at high risk of developing future thromboembolic events. Recurrence remains frequent despite the use of oral anticoagulants, which significantly reduces the risk of thromboembolism.
authors.affiliation: Clinical Cardiology, Thrombosis Center, University Hospital, Padova, Italy. vittorio.pengo@unipd.it
authors.forename: V
authors.initials: V
authors.lastname: Pengo
authors.forename: A
authors.initials: A
authors.lastname: Ruffatti
authors.forename: C
authors.initials: C
authors.lastname: Legnani
authors.forename: P
authors.initials: P
authors.lastname: Gresele
authors.forename: D
authors.initials: D
authors.lastname: Barcellona
authors.forename: N
authors.initials: N
authors.lastname: Erba
authors.forename: S
authors.initials: S
authors.lastname: Testa
authors.forename: F
authors.initials: F
authors.lastname: Marongiu
authors.forename: E
authors.initials: E
authors.lastname: Bison
authors.forename: G
authors.initials: G
authors.lastname: Denas
authors.forename: A
authors.initials: A
authors.lastname: Banzato
authors.forename: S
authors.initials: S
authors.lastname: Padayattil Jose
authors.forename: S
authors.initials: S
authors.lastname: Iliceto
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000368:Aged; D017153:Antibodies, Anticardiolipin / Q000097:blood; D017152:Antibodies, Antiphospholipid / Q000097:blood*; D000925:Anticoagulants / Q000008:administration & dosage / Q000009:adverse effects; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000401:mortality; D015415:Biomarkers / Q000097:blood; D002388:Catastrophic Illness; D018450:Disease Progression; D005260:Female; D006470:Hemorrhage / Q000139:chemically induced; D006801:Humans; D015994:Incidence; D007558:Italy; D053208:Kaplan-Meier Estimate; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000401:mortality; D016016:Proportional Hazards Models; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D054556:Venous Thromboembolism / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000401:mortality; D053482:beta 2-Glycoprotein I / Q000276:immunology
chemical_list: D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D015415:Biomarkers; D016682:Lupus Coagulation Inhibitor; D053482:beta 2-Glycoprotein I