K. A. Breen (1), P. Seed (2), K. Parmar (1, 2), G. W. Moore (1), S. E. Stuart-Smith (1), B. J. Hunt (1, 2)
(1) Guy’s and St.Thomas’ NHS Foundation Trust, London, UK; (2) King’s College, London, UK
The antiphospholipid syndrome (APS) is the association of thrombosis and recurrent pregnancy loss and/or pregnancy morbidity with persistent antiphospholipid antibodies (aPL). Increased complement activation has been implicated in the pathogenesis of APS in animal models. It was our objective to evaluate complement activation in patients with aPL or primary antiphospholipid syndrome (PAPS). We measured complement activation products, fragments Bb and C3a-desArg by ELISA in 186 aPL/PAPS patients and 30 healthy controls. All patients with aPL had significantly increased levels of complement activation products. Fragment Bb levels (mean, 95% CI); (thrombotic APS 0.54 units/ml, 0.31–0.83, obstetric APS 0.60 units/ml,0.39–1.02, isolated aPL 0.48 units/ml, 0.29–0.85, overall 0.39 units/ml, 0.33–0.47) and C3a-desArg levels (mean, 95% CI): (thrombotic APS 261 ng/ml, 219–311, obstetric APS 308 ng/ml, 243–391, isolated aPL 258 ng/ml, 193–337, overall 225 ng/ml, 202–251) were significantly higher compared to controls (fragment Bb 0.06 units/ml, 0.03–0.11, C3a-desArg 69 ng/ml, 50–92). There were correlations between Fragment Bb and C3a-desArg levels in all patients with aPL. Receiver operator characteristic (ROC) analysis showed increased fragment Bb and C3a-desArg levels had strong associations with the presence of persistent lupus anticoagulant (area under ROC: Bb 0.89, and C3a-desArg 0.90), dual and triple aPL positivity (Bb 0.71–0.82, C3a-desArg 0.71–0.80) but not with high titre anti-cardiolipin antibodies (Bb 0.62, C3a-desArg 0.65), or anti β2-glycoprotein 1 antibodies (Bb 0.66, C3a-desArg 0.67). Complement activation is present in all patient groups within this large cohort of patients aPL. This suggests it may have a major role in the pathogenesis of APS and merits further study.
antiphospholipid syndrome, Antiphospholipid antibodies, complement activation
F. Bergmann, M. Hempel
Hämostaseologie 2008 28 3: 141-149
Marielle Sanmarco1, Stéphane Gayet1, Marie-Christine Alessi2, Marie Audrain3, Emmanuel de Maistre4, Jean-Christophe Gris5, Philip G. de Groot6, Eric Hachulla7, Jean-Robert Harlé1, Pierre Sié8, Marie-Claire Boffa9
Thromb Haemost 2007 97 6: 949-954
Anti-prothrombin (aPT) and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies and the risk of thrombosis in the antiphospholipid syndrome
A systematic review
S. Sciascia (1, 2), G. Sanna (3), V. Murru (1), D. Roccatello (2), M. A. Khamashta (1, 3), M. L. Bertolaccini (1)
Thromb Haemost 2014 111 2: 354-364
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