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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of primary antiphospholipid syndrome are described. A girl presented with myocardial infarction at the age of 6. afterward developed chorea, livedo reticularis, thrombocytopenia and circulating lupus anticoagulant (LAC). A boy, age 7, had an episode of intracranial hypertension and a deep venous thrombosis of a lower left limb, both recurrent in the following years. A high titer of IgG anticardiolipin antibodies (aCI) was detected. These observations suggest that both LAC and aCI tests should be performed in children with thromboembolic phenomena when the criteria for a definite autoimmune disease are lacking.
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PMID:Primary antiphospholipid syndrome: a report of two pediatric cases. 192 Mar 12

The antiphospholipid syndrome (characterized by the presence of circulating lupus anticoagulants or anticardiolipin antibodies) was first recognized in patients with systemic lupus erythematosus (SLE), but the syndrome can also exist in the absence of SLE. The clinical features include arterial or venous thrombosis, recurrent abortion, neurological problems, and various cutaneous disorders including thrombophlebitis, livedo reticularis, atrophie blanche, leg ulcers, and gangrene. In some cases, antiphospholipid antibodies may play a role with other recognized syndromes characterized by vascular occlusion, such as Sneddon's syndrome (livedo reticularis with cerebrovascular occlusion) and Degos' disease.
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PMID:Antiphospholipid syndrome and cutaneous vasoocclusive disorders. 193 63

A 64-year-old man with both Factor XI deficiency and a lupus anticoagulant who suffered two myocardial infarctions within a 3-month period is described. Although thromboembolic disorders, including myocardial infarction, have been associated with the antiphospholipid syndrome, myocardial infarction in patients with Factor XI deficiency is rare. The potential role of Factor XIa in fibrinolysis is discussed.
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PMID:Myocardial infarction in a patient with factor XI deficiency and a lupus anticoagulant. 193 5

We present a patient with primary antiphospholipid syndrome, as defined by nine pregnancy losses, the presence of lupus anticoagulant (LAC), and the absence of clinical signs and symptoms of autoimmunity. A successful pregnancy was achieved by treatment with low-dose prednisone (15 mg daily) and aspirin (100 mg daily). The patient was followed-up throughout her two last pregnancies and a 6 months postpartum period. Our data indicate that LAC serves as a marker of disease in women with previous pregnancy wastages, and that aspirin-prednisone therapy is beneficial in carefully selected patients.
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PMID:Lupus anticoagulant as a marker of autoimmunity in recurrent pregnancy loss; a case report. 193 7

The mechanism involved in the association between antiphospholipid antibodies and thrombosis or fetal loss remains unclear. We assessed the biosynthesis of thromboxane A2 and prostacyclin in 31 samples from 25 patients with lupus anticoagulant and in 32 controls. The urinary excretion of the major thromboxane metabolite of platelet origin (11-dehydrothromboxane B2) was very significantly increased (P less than .0003) in the patients. In contrast, the urinary metabolite reflecting the vascular production of prostacyclin (2,3-dinor-6-keto-prostaglandin F1 alpha) was much less increased (P less than .02). We found no correlation between the levels of anticardiolipin antibodies and the urinary excretion of 11-dehydro-thromboxane B2. Six patients with elevated urinary 11-dehydrothromboxane B2 were treated with low-dose aspirin (20 mg/d during 7 days). In these patients, there was a close relationship between the extent of inhibition of the thromboxane urinary metabolite (72%) and serum thromboxane B2 (79%). In contrast, the urinary excretion of 2,3-dinor-6-ketoprostaglandin F1 alpha was nearly unchanged (13% reduction). In addition, the F(ab')2 fragments isolated from six patients presenting increased urinary 11-dehydro-thromboxane B2 enhanced the generation of thromboxane B2 (P = .04) and the release of 14C serotonin (P = .009) by normal washed platelets, as compared with F(ab')2 from controls. In summary, our study shows that in patients with lupus anticoagulant, platelet activation may occur without a compensatory increment in the vascular biosynthesis of prostacyclin. This observation may be crucial to cause or reflect an increased risk for thrombosis. In addition, our results may suggest a rationale for antiplatelet agents for the prophylaxis of thrombosis in many patients with the antiphospholipid syndrome.
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PMID:Imbalance of thromboxane/prostacyclin biosynthesis in patients with lupus anticoagulant. 195 77

Anticardiolipin antibodies are autoantibodies clinically associated with hypercoagulability. Systemic thrombosis and thrombosis of the vascular access for haemodialysis coexist with immunoregulation abnormalities in end-stage renal disease (ESRD). The aim of the present study was to analyse the incidence of thrombotic episodes and the presence of anticardiolipin antibodies and lupus anticoagulant in 73 patients with ESRD--51 on haemodialysis and 22 on conservative treatment. Four (18%) patients on conservative treatment had IgG-anticardiolipin, three of them also having lupus anticoagulant. Sixteen (31%) patients on haemodialysis showed IgG-anticardiolipin and 11 (22%) lupus anticoagulant; overall, 19 (37%) patients on haemodialysis had IgG-anticardiolipin and/or lupus anticoagulant. This greater incidence in haemodialysis was associated with a more frequent use of cuprophane membranes (68% versus 34%, P less than 0.05). Six patients with ESRD--one on conservative treatment--met criteria for the diagnosis of primary antiphospholipid syndrome, clinically characterised by thrombosis of the vascular access. IgG-anticardiolipin and/or lupus anticoagulant are frequently found in ESRD and their incidence increases with haemodialysis, probably due to some kind of membrane bioincompatibility. IgG-anticardiolipin and lupus anticoagulant can be associated with thrombotic episodes, being constituents of an ESRD-related antiphospholipid syndrome.
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PMID:Anticardiolipin antibodies and lupus anticoagulant in end-stage renal disease. 195 52

Positive anticardiolipin and lupus anticoagulant tests are not confined to patients with the antiphospholipid syndrome, and the usefulness of these tests in healthy pregnant women is uncertain. This study sought to determine the prevalence of anticardiolipin antibodies and correlation with pregnancy outcome in 1449 pregnant women. Results were compared with 40 patients with the antiphospholipid syndrome. Persistence of positive anticardiolipin antibody tests was also ascertained. Twenty-six of 1449 sera (1.79%) were immunoglobulin G anticardiolipin positive, and 63 (4.3%) were immunoglobulin M anticardiolipin positive. Twenty-three of 26 positive for immunoglobulin G anticardiolipin and 55 of 63 positive for immunoglobulin M anticardiolipin results were low. Anticardiolipin positivity did not correlate with complications or outcome. Immunoglobulin G isotype and level distinguished patients with antiphospholipid syndrome from otherwise healthy women with positive anticardiolipin tests. In healthy pregnant women positive anticardiolipin tests occur infrequently, at low levels, and are rarely associated with adverse pregnancy outcome. This test should be requested only when the antiphospholipid syndrome is suspected.
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PMID:Should anticardiolipin tests be performed in otherwise healthy pregnant women? 141 13

Anticardiolipin antibodies (aCL), one of a group of antiphospholipid antibodies which include the lupus anticoagulant (LA), may occur in association with systemic lupus erythematosus (SLE) and are less commonly detected in other diseases. We retrospectively reviewed the clinical and immunological features of 39 consecutive patients with abnormal aCL identified by one laboratory, to examine the spectrum of neurological disease in those patients without SLE. Fourteen patients in this category are described, 6 of whom did not have evidence of LA. All but 1 presented with neurological symptoms. Stroke and migraine dominated the clinical presentation, but many patients had features to suggest the presence of a hypercoagulable state. This study lends support to the concept of a primary antiphospholipid syndrome.
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PMID:Neurological disease associated with anticardiolipin antibodies in patients without systemic lupus erythematosus: clinical and immunological features. 196 May 45

A 55-year-old woman with a lupus like disease, associated with the lupus anticoagulant, was admitted because of facial edema. Her facial swelling was previously attributed to the steroids she had been taking and they were tapered without improvement. Laboratory tests revealed high titers of anticardiolipin antibodies. Computerized tomography of her chest and Doppler ultrasound examination of her neck veins demonstrated a thrombus in the superior vena cava. She was treated with heparin and was maintained with longterm warfarin therapy with uneventful followup. Superior vena cava obstruction should be added to the long list of thromboembolic complications of the antiphospholipid syndrome.
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PMID:Superior vena cava occlusion in a patient with antiphospholipid antibody syndrome. 157 77

Habitual abortion is sometimes an incurable pathogenetic state. Even more serious to both the patient and gynecologist is that the incidence of repeated reproductive wastage is higher in subsequent pregnancies. We registered more than 1,000 women with repeated spontaneous abortions in this clinic for 5 years and analyzed their pathogenesis for the wastages by several different approaches. One hundred twenty-four women were diagnosed as having a congenital uterine abnormality. Metroplasty was performed in 50 patients, resulting in 82% successful subsequent pregnancies, whereas more than 98% of the pregnancies had terminated in spontaneous abortion before the operation. By measuring anticardiolipin antibody (ACA) and lupus anticoagulant (LA), the present study diagnosed several cases as antiphospholipid syndrome, and successfully pregnant patients were observed and treated with appropriate medications. Fifty-five partners of 54 couples had either a chromosomal abnormality or normal variants, showing a high incidence of spontaneous abortion in each type of abnormality. In the other 311 cases, female partners were treated by the immunotherapy of the husbands' lymphocytes in pre- and postgestational periods. Among them, 200 women became pregnant, and 147 cases successfully maintained the pregnancy. These studies indicate that early diagnosis through comprehensive examinations is essential for effective treatment of curable fetal loss in human reproduction.
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PMID:Current comprehensive therapy of habitual abortion. 205 75


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