The Role of Complement in the Antiphospholipid Syndrome

Author(s):  
J.E. Salmon ◽  
G. Girardi
2021 ◽  
Vol 23 (2) ◽  
Author(s):  
Silvia Rosina ◽  
Cecilia Beatrice Chighizola ◽  
Angelo Ravelli ◽  
Rolando Cimaz

Abstract Purpose of Review Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS). Recent Findings aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. Summary The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.


2016 ◽  
Vol 45 (5) ◽  
pp. 611-616 ◽  
Author(s):  
Sophie I. Mavrogeni ◽  
Petros P. Sfikakis ◽  
George D. Kitas ◽  
Genovefa Kolovou ◽  
Maria G. Tektonidou

2000 ◽  
pp. 419-425 ◽  
Author(s):  
F. M. K. Williams ◽  
G. R. V. Hughes ◽  
R. M. Leach

Author(s):  
Svetlana Jelic ◽  
Dejan Nikolic ◽  
Dragomir Marisavljević ◽  
Ljudmila Stojanovich

2018 ◽  
Vol 56 (6) ◽  
pp. 939-946 ◽  
Author(s):  
Shulan Zhang ◽  
Ziyan Wu ◽  
Wen Zhang ◽  
Jiuliang Zhao ◽  
Gary L. Norman ◽  
...  

AbstractBackground:Increasing evidence has highlighted the role of non-criteria antiphospholipid antibodies (aPLs) as important supplements to the current criteria aPLs for the diagnosis of antiphospholipid syndrome (APS). In this retrospective study, we evaluated the clinical relevance of antibodies to phosphatidylserine/prothrombin (aPS/PT) in Chinese patients with APS.Methods:A total of 441 subjects were tested, including 101 patients with primary APS (PAPS), 140 patients with secondary APS (SAPS), 161 disease controls (DCs) and 39 healthy controls (HCs). Serum IgG/IgM aPS/PT was determined by ELISA.Results:The levels of IgG/IgM aPS/PT were significantly increased in patients with APS compared with DCs and HCs. IgG and IgM aPS/PT were present in 29.7% and 54.5% of PAPS, and 42.1% and 53.6% of SAPS, respectively. For diagnosis of APS, IgG aCL exhibited the highest positive likelihood ratio (LR+) of 21.60, followed by LA (13.84), IgG aβ2GP1 (9.19) and IgG aPS/PT (8.49). aPS/PT was detected in 13.3% of seronegative PAPS patients and 31.3% of seronegative SAPS patients. LA exhibited the highest OR of 3.64 in identifying patients with thrombosis, followed by IgG aCL (OR, 2.63), IgG aPS/PT (OR, 2.55) and IgG aβ2GP1 (OR, 2.33). LA and IgG aCL were correlated with both arterial and venous thrombosis, whereas IgG aPS/PT and IgG aβ2GP1 correlated with venous or arterial thrombosis, respectively.Conclusions:Our findings suggest that the inclusion of IgG/IgM aPS/PT may enhance the diagnostic performance for APS, especially in those in whom APS is highly suspected, but conventional aPLs are repeatedly negative. In addition, IgG aPS/PT may contribute to identify patients at risk of thrombosis.


Hematology ◽  
2019 ◽  
Vol 2019 (1) ◽  
pp. 426-432 ◽  
Author(s):  
Ecem Sevim ◽  
Rohan Willis ◽  
Doruk Erkan

Abstract Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition (eculizumab), mechanistic target of rapamycin inhibition (sirolimus), vascular endothelial cell modulation (defibrotide), statins, and traditional rheumatologic disease–modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide).


Placenta ◽  
2019 ◽  
Vol 83 ◽  
pp. e86-e87
Author(s):  
Carlos Mario Rodríguez Colorado ◽  
Manuela Velasquez Berrío ◽  
Diana Carolina Rúa Molina ◽  
Angela María Álvarez Gomez ◽  
Angela Patricia Cadavid Jaramillo

2015 ◽  
Vol 14 (11) ◽  
pp. 1066-1071 ◽  
Author(s):  
M. Garcia-Carrasco ◽  
C. Mendoza-Pinto ◽  
S. Macias-Diaz ◽  
F. Vazquez de Lara ◽  
I. Etchegaray-Morales ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document