title: The global anti-phospholipid syndrome score in primary APS.
abstract: OBJECTIVE
  The aim of this study was to evaluate the clinical relevance of the global APS score (GAPSS) in a cohort of primary APS patients.
  
  
  METHODS
  This study included 62 consecutive patients with primary APS. Data on clinical manifestations, conventional cardiovascular risk factors and aPL profile were collected. The GAPSS was calculated for each patient by adding together the points corresponding to the risk factors, based on a linear transformation derived from the β regression coefficient as follows: 3 for hyperlipidaemia, 1 for arterial hypertension, 5 for aCL IgG/IgM, 4 for anti-β2 glycoprotein I IgG/IgM, 3 for aPS-PT IgG/IgM and 4 for LA.
  
  
  RESULTS
  Higher GAPSS values were seen in patients who experienced thrombosis alone when compared with those with pregnancy loss alone [11.5 (S.D. 4.6) and 8.7 (S.D. 3.2), P = 0.04]. Patients with both thrombosis and pregnancy loss showed higher GAPSS than those with pregnancy loss alone [12.5 (S.D. 4.6) vs 8.7 (S.D. 3.2), P = 0.02]. Higher GAPSS values were also shown after subgrouping for the site of thrombosis when compared with pregnancy loss alone [12.2 (S.D. 5.2) for arterial thrombosis, 12.0 (S.D. 4.0) for venous vs 8.7 (S.D. 3.2), P = 0.02 and P = 0.04, respectively]. Patients with thrombotic recurrences showed higher GAPSS values when compared with those without recurrence [13.7 (S.D. 3.1) vs 9.4 (S.D. 3.9), P = 0.02]. This was also seen when comparing recurrences vs no recurrences independently of the site of the thrombotic event [13.9 (S.D. 3.6) vs 11.0 (S.D. 4.3), P = 0.01 for arterial and 13.6 (S.D. 2.18) vs 8.91 (S.D. 3.6), P < 0.01 for venous thrombosis]. GAPSS values ≥11 were strongly associated with a higher risk of recurrence [odds ratio (OR) 18.27 (95% CI 3.74, 114.5) for a cut-off of 11, OR 20.64 (95% CI 3.92, 185.92) for a cut-off of 12 and 21.64 (95% CI 3.89, 189.56) for a cut-off of 15]. GAPSS values ≥11 seemed to have the best risk accuracy in terms of sensitivity and specificity.
  
  
  CONCLUSIONS
  The GAPSS is demonstrated to be a valid tool for a substantial improvement in risk stratification for thrombosis in primary APS.
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK. Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK.
authors.forename: Savino
authors.initials: S
authors.lastname: Sciascia
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK. Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Sanna
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK.
authors.forename: Veronica
authors.initials: V
authors.lastname: Murru
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK.
authors.forename: Dario
authors.initials: D
authors.lastname: Roccatello
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK. Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK.
authors.forename: Munther A
authors.initials: MA
authors.lastname: Khamashta
authors.affiliation: Graham Hughes Lupus Research Laboratory, Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, London, UK, Centro di Ricerche di Immunologia Clinica ed Immunopatologia e Documentazione su Malattie Rare (CMID), Università di Torino, Turin, Italy and Louise Coote Lupus Unit, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, London, UK. maria.bertolaccini@kcl.ac.uk.
authors.forename: Maria Laura
authors.initials: ML
authors.lastname: Bertolaccini
mesh_terms: D000022:Abortion, Spontaneous / Q000453:epidemiology; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis*; D002318:Cardiovascular Diseases / Q000453:epidemiology*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006949:Hyperlipidemias / Q000453:epidemiology; D006973:Hypertension / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D013927:Thrombosis / Q000453:epidemiology
keywords: Hughes syndrome; anti-phospholipid antibodies; pregnancy loss; prothrombin; thrombosis