Presentation Details
| Understanding Primary Antiphospholipid Syndrome: Correlating Antiphospholipid Antibodies Profile and Titers with Thrombotic and Hematological Activity Amaya Llorente-Chávez, Rodrigo Figueroa-Méndez, Gabriela A.Hernández-Molina, Alfonso Orozco-Collazo . Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico |
Abstract
Background: Thrombotic and hematological manifestations are common in primary antiphospholipid syndrome (pAPS) and little is known regarding the differences of the antibodies profile. Aim: To evaluate the coventional and extended profile of antiphospholipid antibodies in pAPS with thrombotic or hematological phenotype. Methods: A retrospective study included patients with pAPS, diagnosed according to the Sapporo criteria with follow-up at third level of care in Mexico City from 1990 to 2021. Descriptive statistics, chi-squared test, Mann-Whitney U test, and logistic regression were performed. Results: A total of 97 patients with pAPS were included; 73.2% were women with a mean age of 33.9 +11.5 years and a mean follow-up of 132 months (24-360). We found that 77% of patients presented thrombotic and 68% hematological manifestations. Patients suffered a mean of 1 (1-3) thrombotic event, being the most common type venous trombosis (VT) in 37.1%, followed by a combination of arterial and venous thrombosis (AVT) in 27.8% and the minority arterial thrombosis (AT) in 12.4%. Those who had AVT had higher titers of anticardiolipin (aCL) IgG and IgM in 34.6 and 9.6, respectively (p = 0.031), anti-β2 glycoprotein-I (aβ2GPI) IgM in 6.6 (p = 0.01) and anti-phosphatidyl serine/prothrombin (aPS/PT) IgG in 113.8 (p = 0.044) from the three groups. Patients with VT compared to those with AT had higher titers of aCL IgG in 9.6 vs 6.6 (p = 0.031), IgM in 10.8 vs 7.25 (p = 0.005), and aβ2GPI IgM in 5.7 vs 4.4 (p = 0.01). Cases with AT had higher titers of aPS/PT IgG in 55.4 (p = 0.044). Regarding the hematological activity; 63.9% patients had thrombocytopenia, most commonly being severe. A total of 59.7% had a triple marker. In comparison with patients without thrombocytopenia, 69.4% presented positive lupus anticoagulant vs. 48.6% (p = 0.043) and OR 2.5 (CI 95% 1.06-6.1). An 83.9% had positive aCL IgG vs. 62.9% (p = 0.019) and OR 3.21 (CI 95% 1.19-8.63) with a higher titer of 19.4 vs. 6.1 (p = 0.017). Also higher titers of aβ2GPI IgM of 6.8 vs. 5.2 (p = 0.019) as of aPS/PT IgM of 52.8 vs. 16.8 (p = 0.004). A 13.4% of patients had autoimmune hemolytic anemia, with higher aβ2GPI IgM titers of 7.1 vs. 5.9 when compared with those who did not present it (p = 0.026) and 77% had thrombosis. Those without hematological activity had more thrombosis (p <0.001). When comparing the groups with and without thrombosis, we observed that those with thrombosis had lower titers of aβ2GPI IgM in 5.8 vs. 7.4, p = 0.036 and aCL IgM in 9.4 vs 12.8, p = 0.09. They had higher aPS/PT titers in 61.2 vs. 12.1, p = 0.021. Conclusion: Differences were found regarding the titer and positivity of antibodies between the thrombotic or hematological phenotype; and also between the three groups of thrombosis. Knowing the serology of pAPS is useful to promptly identify patients with hematological or thrombotic activity as well of complications. In our knowledge this is one of the few studies that has compared the antibodies profile of the different phenotypes.
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No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the author.