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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In patients with systemic lupus erythematosus (SLE) both a haemorrhagic diathesis and a tendency to thrombosis of the venous and arterial vessels can be observed. In the course of the disease, thrombosis of the leg or pelvic veins developed in 20 per cent of 188 patients. The levels of alpha 2-plasmin inhibitor, plasminogen, fibronectin and of factor VIII complex were increased in patients with SLE compared with a control group. Fifty per cent of the patients showed no increase in fibrinolytic activity after venous occlusion measured with the fibrin plate method. This suggests a reduced fibrinolytic capacity in SLE probably caused by alteration of the endothelial cells through immune complex vasculitis. In addition, the lupus anticoagulant and an acquired antithrombin III deficiency in nephrotic syndrome in SLE are to be considered thrombophilic mechanisms. In the individual case there is an overlapping of hyper- and hypocoagulability.
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PMID:[Status of fibrinolysis in systemic lupus erythematosus]. 242

The coagulation system can be considered as a balance in which clotting and fibrinolysis have to be in a state of equilibrium. Increased fibrin formation or decreased fibrinolysis can predispose to thromboembolic diseases. Derailments in the clotting system leading to thrombosis center around the regulatory mechanisms, antithrombin III, protein C, protein S and possibly heparin cofactor II. Many cases of congenital or acquired deficiencies or abnormalities or antithrombin III, protein C and S have been described, all predisposing to thrombotic events. Alterations of the fibrinolytic system can also be associated with thromboembolisms. In particular, abnormalities of plasminogen, tissue plasminogen activator release and elevated tissue plasminogen activator inhibitor levels seem to be associated with thromboses. Conceivably also factor XIIa (Hageman factor) and prekallikrein deficiencies, when associated with thrombosis, exert their mechanism through the fibrinolytic system. Finally, about 50% of patients with lupus anticoagulant seem to suffer from thromboembolic disorders. The pathophysiology of this particular association is not known with certainty. Undoubtedly, there will be more disturbances discovered in the hemostasis system that are associated with increased intravascular fibrin formation. The understanding of these derailments is at this time only in its earliest stages of development.
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PMID:Pathophysiology of thrombophilic states. 246 10

We retrospectively evaluated 66 patients younger than 40 years of age who presented with acute nonhemorrhagic cerebral infarction (n = 63) or transient ischemic attacks (n = 3) to determine the possible etiology and long-term outcome at a mean follow-up interval of 3 years after initial presentation. A probable cause for the stroke was identified in 24 patients (36%); this group included one woman with a history of recurrent spontaneous abortions and a positive test for the presence of the lupus anticoagulant. We performed detailed hemostatic investigations at follow-up in 38 (90%) of the remaining 42 patients in whom the cause of the stroke was unknown or uncertain; results of the basic hemostatic screening tests (including that for fibrinogen) were uniformly normal. All 38 patients demonstrated a normal fibrinolytic response as measured by tissue plasminogen activator release to a standard venous occlusion stress test; concentration of the inhibitor of tissue plasminogen activator was not increased. No abnormalities in the concentrations of the inhibitory proteins C or S or antithrombin III were identified, and none of the 38 patients had evidence of a lupus anticoagulant. Neurologic recovery was complete or the residual disability mild in 46 of 59 (78%) patients. Overall prognosis was excellent and independent of whether a precipitating factor for the stroke could be identified.
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PMID:Etiology, prognosis, and hemostatic function after cerebral infarction in young adults. 249 81

Lupus anticoagulant, concentrations of anticardiolipin antibodies, antithrombin III, plasminogen, (free) protein S, protein C, prothrombin, platelet counts, and bleeding times were determined in 74 lupus patients (58 with systemic lupus erythematosus; 16 with lupus-like disease) to establish the presence of risk factors for thrombosis in these patients. Of the variables evaluated, lupus anticoagulant had the strongest association with a history of thrombosis. Both positive anticardiolipin antibody concentrations and the presence of (mild) thrombocytopenia were significantly associated with a history of thrombosis and the presence of lupus anticoagulant. Reduced concentrations of antithrombin III, plasminogen, (free) protein S, and protein C were found in some patients but were not associated with either thrombosis or lupus anticoagulant. Mean concentrations of total protein S were significantly lower in patients with thrombosis than in those without and in patients with lupus anticoagulant than in those without. The antigenic concentration of prothrombin was reduced in 3/74 (4%) lupus patients. These three patients had lupus anticoagulant but no history of thrombosis, which suggests that a low prothrombin concentration protects patients with lupus anticoagulant from the development of thrombosis. A prolonged bleeding time was associated with the presence of lupus anticoagulant but not with a history of thrombosis. Analysis by stepwise logistic regression did not disclose additional risk factors for thrombosis in lupus patients with lupus anticoagulant. Increased antithrombin III concentrations and decreased free protein S concentrations are often found in lupus patients, unrelated to lupus anticoagulant or thrombosis.
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PMID:Risk factors for thrombosis in lupus patients. 251 63

Heparin is a complex polysaccharide, consisting of repeating dissacharide subunits, which exerts its anticoagulant effect by potentiating the inhibition of activated clotting proteins by the naturally occurring inhibitor antithrombin III. The primary indication for its use is venous thromboembolic disease where an average 20,000 to 40,000 USP units are initially administered by constant infusion over 24 hours to prevent extension of an established thrombus. Subsequent treatment is based on the therapeutic response, usually monitored by a global clotting test such as the activated partial thromboplastin time (APTT). The chromogenic assay based on heparin-induced inhibition of activated factor X (Xa) is a particularly useful alternative monitoring test where thrombosis complicates pregnancy or is associated with the lupus anticoagulant. Subcutaneous heparin has also been used for the primary treatment of venous thrombosis but is more frequently used either for primary or secondary prophylaxis. Primary prophylaxis schedules usually employ a low dose (5000 units) administered 8- or 12-hourly without anticoagulant control, but a titrated subcutaneous heparin regimen has been successfully reported in elective hip surgery. Although arterial thrombosis is primarily initiated by platelet aggregates forming in vivo, heparin is commonly administered for acute arterial thromboembolism including peripheral arterial occlusion and repeated transient cerebral ischaemic events. The potential efficacy of heparin in disseminated intravascular consumption (DIC) remains to be firmly established, but is indicated where symptomatic thrombotic complications occur. Thrombocytopenia and haemorrhagic side effects may complicate heparin therapy, but bleeding complications may be minimised with the development of modified low molecular weight heparin which is currently undergoing clinical trial.
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PMID:Heparin 1986. Indications and effective use. 351 Jan 15

Some molecular defects of components of the coagulation or fibrinolytic system are associated with thromboembolism. One possibility is that physiologic inhibitors of the coagulation system have an abnormal function e.g. protein C, protein S, antithrombin III and cofactor II of heparin. Also a hindered activation of the fibrinolytic system may predispose to thrombosis; the impaired activation may be due to deficient synthesis and/or release of tissue-plasminogen activator, an increased level of its inhibitor or a functional defect of the plasminogen molecule. A few cases of congenital dysfibrinogenemia have been described in which the functional defects of the molecule are held responsible for recurrent thrombosis. An acquired thrombotic disorder is due to the presence of immunoglobulins which prolongs phospholipid-dependent coagulation by binding to epitopes of some phospholipids. This so-called lupus anticoagulant was originally described in patients with systemic lupus erythematosus but is a misnomer as it is more frequently encountered in patients without lupus.
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PMID:[Molecular defects of coagulation factors and of the fibrinolytic system associated with thromboembolism]. 354 55

The authors report their experience with 45 cases of inferior vena cava thrombosis. Diagnosis was delayed for an average of 55 days. One-third of cases were revealed by an embolic complication. Inflammatory diseases were the most common causes (Behcet disease: seven cases, systemic lupus erythematosus: 5 cases). Malignancies accounted for 20% of cases. Abnormalities of coagulation were uncommon: antithrombin III deficiency in one patient and protein C deficiency in another. Estrogen-progestogen combinations could be incriminated in 4 cases. Outcome was fatal in 20% of cases, usually as a result of the underlying disease. Functional status was good in two-thirds of patients without malignancy followed up for an average of 27 months. In 14 patients a clip was inserted to ensure total (3 cases) or partial (11 cases) interruption of vena cava blood flow because of a free thrombus and/or recurrent pulmonary embolism. Three patients had thrombectomy. After clip insertion two embolisms were recorded, one of which occurred in the immediate post-operative period.
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PMID:[Inferior caval syndromes. Apropos of 45 cases]. 632 Apr 31

Plasma antithrombin III (AT III) was studied in 39 patients with systemic lupus erythematosus (SLE) and in 12 patients with other connective tissue disorders. AT III was measured immunologically by the Mancini method as well as by functional assay using thrombin and the chromogenic substrate, chromozyn TH (Boehringer). Reduced AT III activity was found in 17 patients; 8 had thrombosis. In 6 patients low AT III correlated with disease exacerbations and 2 had systemic vasculitis. No significant correlation could be demonstrated between low AT III levels and thromboembolic disease. A marked variation of functional AT III activity was observed in 30 patients in whom the presence of the lupus anticoagulant was demonstrated. The significance of this association is discussed.
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PMID:Antithrombin III in systemic lupus erythematosus. 644 39

Coagulation studies were performed in 112 consecutive patients with systemic lupus erythematosus (SLE). Abnormalities of haemostatic function occurred frequently and 96 abnormalities occurred in 64 of 112 (57 per cent) patients. Eighteen patients (16 per cent) had thrombocytopenia, 19 (16.9 per cent) had circulating anticoagulants and 24 had decreased antithrombin III levels. Abnormalities of fibrinogen were found in 28 patients (23 per cent), and abnormalities of platelet factor 3 and 4, indicating in vivo platelet activation occurred in seven patients. In 25 patients two or more abnormalities were detected simultaneously. No haemostatic abnormalities were detected in any of the 50 healthy volunteers who served as controls. Only one patient with thrombocytopenia had petechiae. None of the other patients, even those with multiple defects bled significantly, but several patients had vasculitis and/or phlebitis. There was no correlation between disease activity of SLE and the presence of haemostatic abnormalities, nor was there an association between these abnormalities and specific clinical haematologic manifestations.
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PMID:Haemostatic abnormalities in systemic lupus erythematosus. 664 51

The increased frequency of thromboembolic events in patients with systemic lupus erythematosus (SLE) has been attributed to reduced or dysfunctional antithrombin III (At-III). We analysed At-III values, measured by three different assay techniques, in SLE patients, patients with rheumatoid arthritis, and normal and hospitalised controls. In addition, attempts were made to correlate At-III activities of SLE patients with specific clinical and serological parameters such as disease activity, renal involvement, previous thrombosis, degree of proteinuria, and serum complement concentrations. Our results failed to show a significantly reduced At-III in SLE with any method. At-III titres did not correlate with disease activity, concentrations of serum complement or albumin (both only minimally reduced in most patients), or a previous history of thrombosis. At-III deficiency does not appear to be an inherent feature of SLE, and reduced activities should only be anticipated when there are specific aetiological factors present, such as massive proteinuria, extensive hepatic disease, or active thrombosis.
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PMID:Relation between antithrombin III and clinical and serological parameters in systemic lupus erythematosus. 682 82


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