Prevalence of Annexin A5 Resistance in Children and Adolescents with Rheumatic Diseases

2011 ◽  
Vol 39 (2) ◽  
pp. 382-388 ◽  
Author(s):  
DAWN M. WAHEZI ◽  
NORMAN T. ILOWITE ◽  
SWAPNIL RAJPATHAK ◽  
JACOB H. RAND
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Children And Adolescents ◽  
Rheumatic Diseases ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Anticoagulant Activity ◽  
Underlying Mechanism ◽  
Annexin A5 ◽  
Systemic Lupus

Objective.The underlying mechanism(s) by which antiphospholipid antibodies (aPL) result in thrombosis remains poorly understood. A significant body of evidence has evolved to support the hypothesis that antibody-mediated disruption of an annexin A5 anticoagulant shield may play a role in the pathogenesis; this proposed mechanism has not been previously studied in children.Methods.We investigated the association between aPL and resistance to annexin A5 anticoagulant activity in 90 children with a variety of rheumatic diseases using a novel mechanistic assay, the annexin A5 resistance assay (A5R).Results.Patients with a diagnosis of primary aPL syndrome, systemic lupus erythematosus, and mixed connective tissue disease demonstrated lower mean A5R levels (p = 0.030), higher prevalence of positive aPL (p < 0.001), and more thrombotic events (p = 0.014) compared to those with other diagnoses. Patients with persistently positive aPL had significantly lower mean A5R compared to patients with no aPL (mean A5R = 203% ± 44% vs 247% ± 35%; p < 0.001), whereas patients with transient aPL did not. Patients with thrombosis had lower A5R levels compared to those without thrombosis (mean A5R = 207% ± 36% vs 237% ± 46%; p = 0.048).Conclusion.Children and adolescents with rheumatic diseases and persistent aPL have reduced annexin A5 anticoagulant activity, whereas transient, nonpathogenic aPL have less effect on annexin A5 activity.

scholarly journals Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus

2003 ◽  
Vol 58 (3) ◽  
pp. 157-162 ◽  
Author(s):  
Lúcia Maria de Arruda Campos ◽  
Maria Helena B. Kiss ◽  
Élbio A. D'Amico ◽  
Clóvis Artur Almeida Silva
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Children And Adolescents ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Anticardiolipin Antibodies ◽  
Systemic Lupus ◽  
Severity Scores ◽  
And Behavior

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2% and 75.4% respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters.

scholarly journals Fibrinolytic Potential and Antiphospholipid Antibodies in Systemic Lupus Erythematosus and Other Connective Tissue Disorders

1992 ◽  
Vol 68 (05) ◽  
pp. 516-520 ◽  
Author(s):  
M Jurado ◽  
J A Páramo ◽  
M Gutierrez-Pimentel ◽  
E Rocha
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Venous Occlusion ◽  
Tissue Type ◽  
Control Group ◽  
Connective Tissue Disorders ◽  
Systemic Lupus ◽  
Pai 1

SummaryWe studied the fibrinolytic response before and after venous occlusion (VO) in 30 patients with systemic lupus erythematosus (SLE), 25 with rheumatoid arthritis (RA) and 25 with different connective tissue disorders. Results were compared in patients with and without antiphospholipid antibodies (APA) and a history of either thrombosis or abortions. Before occlusion plasma levels of tissue-type plasminogen activator (t-PA) antigen and its inhibitor (PAI-1) were significantly higher in the patient group (p <0.001). After occlusion, a low fibrinolytic activity on fibrin plates (p <0.005) was observed in the same group. t-PA capacity and t-PA release were similar in relation to controls. The plasma PAI-1 activity was significantly elevated in each group of patients (p <0.005) as compared to the control group. No significant differences with respect to t-PA and PAI-1 were observed in patients as to the presence or absence of thrombosis. There was also no correlation between the fibrinolytic changes and the presence of APA. It is concluded that an impairment of the fibrinolytic system, mainly related to increased PAI-1 levels, is present in most patients with connective tissue disorders, although these changes did not correlate with the presence of APA or the incidence of thrombosis.

The Coagulation Abnormalities in Systemic Lupus Erythematosus

1968 ◽  
Vol 20 (03/04) ◽  
pp. 457-464 ◽  
Author(s):  
L Gonyea ◽  
R Herdman ◽  
R. A Bridges
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Ammonium Sulfate ◽  
Lupus Erythematosus ◽  
Anticoagulant Activity ◽  
Species Specificity ◽  
Sensitive Assay ◽  
Systemic Lupus ◽  
Coagulation Abnormalities

SummaryAn anticoagulant occurring in 4 of 6 patients with SLE has been demonstrated by a sensitive assay utilizing an ammonium sulfate fraction of serum. The anticoagulant functions as an inhibitor of the activation of prothrombin. No species specificity was demonstrable. The inhibitor behaves clinically and chromatographically as an immunoglobulin, although an attempt to demonstrate directly the antibody nature of the inhibitor was not successful.A severe, apparently independent, decrease in the level of prothrombin was observed in the patient with hemorrhagic symptoms. In contrast to the anticoagulant activity, the low prothrombin has persisted during treatment.

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