Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSPRADIN, MassimoUGOLINI-LOPES, Michelle RemiaoSCIASCIA, SavinoANDRADE, Danieli2018-09-132018-09-132018SEMINARS IN ARTHRITIS AND RHEUMATISM, v.48, n.1, p.117-120, 20180049-0172https://observatorio.fm.usp.br/handle/OPI/28380Objectives: Extra-criteria manifestations of antiphospholipid syndrome (APS) might impact on prognosis and morbidity of the disease. In this study, we aimed to evaluate a population of patients with primary APS (PAPS) whether the extra-criteria manifestations were more frequently found in subjects with higher adjusted Global APS Score (aGAPSS) values when compared to patients with thrombotic and/or obstetric APS (""criteria"" manifestations) only. Methods: Clinical records were analyzed to retrieve extra-criteria manifestation of APS, cardiovascular risk factors and antiphospholipid antibodies profile. The aGAPSS was calculated by adding the points, as follows: 3 for hyperlipidaemia, 1 for arterial hypertension, 5 for anticardiolipin antibodies IgG/IgM, 4 for anti-beta 2 glycoprotein I IgG/IgM, and 4 for lupus anticoagulant. Results: This retrospective multicenter study included 89 consecutive PAPS [mean age 43.1 (S.D. +/- 12.9), female 67%, 52% arterial and 65% venous]. Twenty-seven patients (30.3%) had a history of livedo, 19 (21.3%) had a history of confirmed thrombocytopenia, 3 (3.4%) had biopsy-proven antiphospholipid antibodies (aPL)-related nephropathy and 3 (3.4%) had a history of valvulopathy. Patients with extra criteria manifestations presented a mean aGAPSS significantly higher [mean 10.30 (S.D. +/- 3.57, range: 4-17) vs mean 8.16 (S.D. +/- 3.52;range: 4-16, p = 0.005). When comparing patients with and without extra-criteria manifestations, the first group had significantly higher incidence of anti-beta 2GPI antibodies positivity (59% and 33%, respectively, p = 0.015), double aPL positivities (53% and 31%, respectively, p = 0.034), cerebrovascular events history (52% and 24%, respectively, p = 0.007) and arterial hypertension (52% and 24%, respectively, p = 0.007). Conclusions: Our results suggest that patients with higher aGAPSS, might be at higher risk for developing extra-criteria manifestations of APS and should therefore undergo a thorough laboratory and instrumental evaluation.engrestrictedAccessAntiphosphospholipid syndromeAPSLivedoThrombocytopeniaGAPSSaGAPSSRisk scoreThrombosissyndrome scoresyndrome apscohortupdatevalidationthrombosisgapssarticleCopyright W B SAUNDERS CO-ELSEVIER INC10.1016/j.semarthrit.2017.12.006Rheumatology1532-866X