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.

Coagulation and Fibrinolysis Study in Systemic Lupus erythematosus: Haematological, Urinary and Tissue Parameters

1981 ◽  
Vol 46 (03) ◽  
pp. 575-580 ◽  
Author(s):  
P Stratta ◽  
C Canavese ◽  
P Valmaggia ◽  
M Rotunno ◽  
E Levi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Immune Complexes ◽  
Acute Stage ◽  
Systemic Lupus ◽  
Immunological Activity ◽  
Coagulation Abnormalities ◽  
Coagulation And Fibrinolysis

SummaryHaematochemical, urinary and tissue parameters were examined in the elaboration of the coagulation and fibrinolysis profile in 33 cases of systemic lupus erythematosus in different stages of the disease. Coagulation abnormalities varied from hypo- to hyper-coagulabitity, these being often associated in the same patient, either simultaneously or at different stages of the disease. Activation of coagulation, closely related to the immunological activity of the disease, was present in 80% cases in the acute stage, and 36% of those in the remission stage. The lupus-like anticoagulant was not much involved, and platelets were the prime figures in the haemostatic abnormalities of lupus, those being the preferred target of direct antibody activities, or possibly of immune complexes as well. Activation of the coagulatory cascade is not uncommonly accompanied by a thrombophilic tendency coupled with signs of consumption, this being the expression of a continuously stimulated haemostatic balance.

scholarly journals A Plasma Coagulation Defect in Systemic Lupus Erythematosus Arising from Hypoprothrombinemia Combined with Antiprothrombinase Activity

Blood ◽  
1960 ◽  
Vol 15 (2) ◽  
pp. 212-227 ◽  
Author(s):  
SAMUEL I. RAPAPORT ◽  
SARA BETH AMES ◽  
BARBARA J. DUVALL
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Anticoagulant Activity ◽  
The Other ◽  
Severe Bleeding ◽  
Systemic Lupus ◽  
Plasma Coagulation ◽  
Complex Plasma ◽  
Coagulation Defect

Abstract A patient has been described with systemic lupus erythematosus and severe bleeding. Her bleeding was associated with a complex plasma coagulation disturbance consisting of profound hypoprothrombinemia plus an anticoagulant active against formed blood and tissue prothrombinase. The problem of the recognition of hypoprothrombinemia in the presence of this type of anticoagulant has been discussed in detail. An analysis of previously reported cases reveals that our patient’s findings are not unique. It appears that the plasma coagulation disturbances of systemic lupus erythematosus characteristically result from a mixture of anticoagulant activity and true hypoprothrombinemia. In an individual patient one or the other may predominate.

Inhibition of Lupus Anticoagulant Activity by Hexagonal Phase Phosphatidylethanolamine in the Presence of Prothrombin

1998 ◽  
Vol 80 (12) ◽  
pp. 936-941 ◽  
Author(s):  
Marion Tannenbaum ◽  
Carolyn Neville ◽  
Paul Fortin ◽  
Joyce Rauch
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Plasma Proteins ◽  
Anticoagulant Activity ◽  
Hexagonal Phase ◽  
Annexin V ◽  
Systemic Lupus ◽  
Glycoprotein I ◽  
No Inhibition

SummaryWe have previously demonstrated that lupus anticoagulant antibodies from patients with systemic lupus erythematosus (SLE) specifically recognize hexagonal (II) phase phosphatidylethanolamine (PE), but not bilayer PE (Thromb Haemost 1989; 62: 892). In those studies, the involvement of proteins in this recognition was not evaluated. To address this issue, we have isolated IgG lupus anticoagulant antibodies from the plasma of SLE patients and evaluated the inhibition of lupus anticoagulant activity by hexagonal (II) phase PE in the presence and absence of purified plasma proteins. All six of the IgG lupus anticoagulant antibodies tested were inhibited by hexagonal (II) phase PE in the presence, but not the absence, of human prothrombin. In contrast, little or no inhibition was observed with prothrombin alone or with PE in combination with either β2-glycoprotein I or annexin V. These data indicate that, for certain lupus anticoagulant antibodies, inhibition by hexagonal (II) phase PE is dependent on prothrombin, suggesting that these antibodies recognize a complex of PE and prothrombin.

scholarly journals Monoclonal IgG anticoagulants delaying fibrin aggregation in two patients with systemic lupus erythematosus (SLE)

Blood ◽  
1978 ◽  
Vol 52 (5) ◽  
pp. 1037-1046
Author(s):  
DK Galanakis ◽  
EM Ginzler ◽  
SM Fikrig
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Self Assembly ◽  
Anticoagulant Activity ◽  
Rabbit Serum ◽  
Distinct Region ◽  
Thrombin Time ◽  
Systemic Lupus ◽  
Fibrinogen Molecule ◽  
Anticoagulant Property

There is paucity of information regarding the prolonged plasma thrombin time known to occur in some patients with systemic lupus erythematosus. Detailed investigations of plasma from two such patients disclosed that IgG accounted for this defect in each case. IgG isolated from plasma of either patient possessed the property of delaying fibrin aggregation and prolonging the clotting times of fibrinogen. Preincubation of IgG from either patient with anti-IgG or anti-Fab (rabbit) serum abolished this anticoagulant property. Moreover, the anticoagulant IgG from the first patient was neutralized with anit-k chain and anti-IgG3, that from the second patient with anti-lambda chain and anti-IgG1 serum. These anticoagulants were also dissimilar with respect to their interactions with fibrin(ogen). IgG from the first patient had no anticoagulant activity against fibrin(ogen) species lacking intact Aalpha chains. IgG from the second patient displayed undiminished anticoagulant effect on such fibrin(ogen) species. We conclude that each anticoagulant interacted with a distinct region(s) on the fibrinogen molecule and that these interactions affect or involve sites that participate in the fibrin self-assembly process.

scholarly journals Coagulation Abnormalities in Fatal Cerebral Lupus Erythematosus1

1981 ◽  
Vol 74 (1) ◽  
pp. 53-55 ◽  
Author(s):  
A Paterson ◽  
W Timperley ◽  
D Slater
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiplatelet Drugs ◽  
Systemic Lupus ◽  
Coagulation Abnormalities ◽  
Cerebral Involvement ◽  
The Brain ◽  
Fibrin Thrombi

Two cases of fatal systemic lupus erythematosus (SLE) with cerebral involvement are described. Both showed widespread microinfarction of the brain secondary to platelet and fibrin thrombi. There was no evidence of vasculitis. Anticoagulants and antiplatelet drugs may have an important role in the management of cerebral SLE.

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 Reversible renal failure in the primary antiphospholipid syndrome--a report of two cases.

1993 ◽  
Vol 4 (1) ◽  
pp. 28-35
Author(s):  
D O Sokunbi ◽  
F Miller ◽  
N K Wadhwa ◽  
E P Nord
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Renal Failure ◽  
Renal Function ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Primary Antiphospholipid Syndrome ◽  
Systemic Lupus ◽  
Coagulation Abnormalities ◽  
Objective Evidence

The primary antiphospholipid/anticardiolipin syndrome is a recently described entity wherein multiorgan thrombotic events occur in the absence of objective evidence of systemic lupus erythematosus. The spectrum of renal involvement remains poorly described. Two patients with coagulation abnormalities consistent with the primary antiphospholipid/anticardiolipin syndrome who developed profound renal insufficiency are reported. Striking microangiopathic lesions were documented on renal biopsy. Renal function improved concomitant to the institution of steroid therapy. Reversible renal failure should be added to the spectrum of clinical manifestations of this entity. The diagnosis of the primary antiphospholipid/anticardiolipin syndrome should be contemplated in individuals with unexplained acute renal failure.

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