Massimo Radin1, Michelle Remião Ugolini-Lopes2, Savino Sciascia3, Danieli Andrade2. 1. Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, and SCDU Nephrology and Dialysis, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy. Electronic address: massimo.radin@unito.it. 2. Hospital dasClinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil. 3. Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, and SCDU Nephrology and Dialysis, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy.
Abstract
OBJECTIVES: 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-β2 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-β2GPI 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.
OBJECTIVES: 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-β2 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-β2GPI 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.
Authors: C Barbati; L Stefanini; T Colasanti; E Cipriano; A Celia; G Gabriele; M Vomero; F Ceccarelli; F R Spinelli; A Finucci; M Speziali; G Orso; D P E Margiotta; F Conti; F Violi; A Afeltra; G Valesini; C Alessandri Journal: Arthritis Res Ther Date: 2019-07-01 Impact factor: 5.156