Gene/Protein Disease Symptom Drug Enzyme Compound
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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 order to assess the prevalence of cardiac involvement in the primary antiphospholipid syndrome (PAS), a syndrome which associates thromboembolism, recurrent abortion, the presence of antiphospholipid antibodies and thrombocytopenia, transthoracic (TTE) and trans-esophageal echocardiography (TEE) was performed in 15 patients, 10 women and 5 men with a mean age of 38.8 +/- 11 years, with the PAS but without systemic lupus erythematosus. The presentation of the PAS was a thrombotic event (6 arterial and 7 venous) in 13 cases, and recurrent abortion in 3 cases. Twelve patients had high anticardiolipin antibody levels (> or = 15 U GPL) and 12 had a raised anti-prothrombinase antibody title. Valvular heart disease was detected in 9 patients (60%) as a valve thickening (> or = 5 mm for the mitral and > or = 3 mm for the aortic valve) or nodule. Mitral regurgitation was observed in 4 cases both on TTE and TEE and was mild in 3 cases and severe in 1 case. Aortic regurgitation was diagnosed in 6 patients, in 3 cases by TTE and in 6 cases by TEE. It was mild in 5 cases and moderate in the other cases. Pericardial effusion was observed in 3 patients (20%), alone in 1 case and associated with valvular disease in the other two cases. No abnormality of left ventricular systolic or diastolic function could be demonstrated. In conclusion, cardiac involvement seems to be common in the PAS, and TEE is a sensitive and accurate method for describing the valvular, especially aortic valve, abnormalities.
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PMID:[Prevalence and description of cardiac involvements in primary antiphospholipid syndrome]. 130 23

In patients with systemic lupus erythematosus (SLE), the synthesis of antibodies to cardiolipin (A-CL) is associated with the development of venous and arterial thromboses localized in minor and middle-sized vessels, in the venous system and capillaries. The determination of various A-CL isotypes may be used to predict thromboses in SLE patients. Overall 210 patients (185 women and 25 men) with a verified diagnosis of SLE were examined. The patients were not screened in accordance with some or other clinical signs of the antiphospholipid syndrome. The control group comprised 100 healthy subjects (donors). The IgG, IgA and IgM isotypes of A-CL were determined by ELISA. Sera of SLE patients showed an increase of the concentration of A-CL of both certain isotypes and their potential combinations. Among A-CL-positive patients, the IgG isotype of A-CL was detected in 75% of cases, the IgM isotype of A-CL in 61%, and the IgA isotype of A-CL in 36% of cases. Thrombotic complications were recorded in 19% of patients. They were induced by hyperproduction of the three combinations of the A-CL isotypes: A-CL IgM, IgM+IgA, and IgG+IgA+IgM. Patients whose sera contained A-CL of all three types at a time were most prone to thrombotic complications. It has turned out that the percentage of SLE patients with thromboses was higher than that of SLE patients without thromboses, starting from the definite A-CL concentration (21 GPL).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[The prediction of thrombosis development in patients with systemic lupus erythematosus: the role of antibodies to cardiolipin]. 145 69

One hundred and two patients suffering from systemic lupus erythematosus were observed over 5-140 months. IgG and IgM anticardiolipin antibodies (aCL) were searched for by ELISA in 448 serum samples, and were found in 88 (86.3%) patients on at least one occasion. Changes of aCL levels and isotypes were recorded in most patients. In patients with medium (20-80 U. GPL and/or MPL) and high (> 80 U. GPL and/or MPL) aCL levels associations with thrombosis, neurological manifestations, cerebrovascular disease, prolonged aPTT, and thrombocytopenia were found. A highly significant statistical association was found between IgG aCL and the occurrence of active disease (p < 0.0001).
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PMID:Longitudinal survey of anticardiolipin antibodies in systemic lupus erythematosus. Relationships with clinical manifestations and disease activity in an Italian series. 147 36

The existence of circulating lupus anticoagulant and anti-cardiolipin antibodies (ACA) has been reported to be associated with recurrent fetal loss. We used an enzyme-linked immunosorbent assay (ELISA) for detection of ACA in plasma samples from 104 women with a history of recurrent fetal loss. The normal range of the ACA level was defined as less than 6 GPL (IgG anti-cardiolipin) units (n = 100 normal plasma samples). Nine women (9.7%) were positive for ACA. The population was divided into two groups on the basis of medical history, and analysis revealed that 42.8% (3/7) of the group of patients with at least one fetal loss in the second or third trimester were positive for ACA; their mean ACA titer was 34 GPL units.
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PMID:Correlation between trimester of fetal wastage and anti-cardiolipin antibody titer. 167 36

We describe the case of a patient with primary familiar antiphospholipid syndrome and acute myocardial infarction. A previously healthy 15-year-old adolescent was admitted with severe chest pain lasting from 1 hour associated with inferoposterolateral ST-segment elevation. The patient received intravenous thrombolysis. A 2-dimensional echocardiogram revealed an area localized in the basal posterolateral left ventricular myocardium, that was akinetic and abnormally thin throughout the cardiac cycle. Peak creatinine kinase level was 1461 U/I. Subsequent electrocardiogram revealed inferoposterior infarction. Plasma anticardiolipin (aCL) IgG antibodies resulted positive (24 U.GPL) in repeated determinations. A dypiridamole echocardiographic test resulted negative. The patient's parents refused cardiac catheterization. He continues to do well at home 28 months after discharge. The patient's sister is affected by primary antiphospholipid syndrome characterized by recurrent abortion, very low platelet count and lupus anticoagulant positivity. Plasma aCL antibodies resulted positive also in the mother who did not have clinical manifestations.
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PMID:[Primary antiphospholipid syndrome with a familial element and myocardial infarct in an adolescent]. 749 21

Anticardiolipin antibodies (aCL) are found in about 40-50% of patients suffering from systemic lupus erythematosus (SLE) and their presence carries an increased risk of thromboembolism. Since there is a high prevalence of nailfold capillary abnormalities in patients with SLE, we studied the relationship between aCL and skin microcirculatory changes or vascular symptoms in 51 consecutive patients with SLE (49 women, 2 men, 34.8 +/- 13.7 years). Twenty-two patients (43.1%) had positive aCL (IgG 22 (5-60) GPL; IgM 5 (3-16.5) MPL; median titre and range) and 12 (54.5%) of them had abnormal capilloscopic findings. By contrast, among the 29 patients without aCL, only six (20.7%) had an abnormal capillaroscopy (P = 0.027). There was no correlation between either aCL or capillaroscopy and Raynaud's phenomenon. These results show a relationship between aCL and nailfold capillary changes in patients with SLE, suggesting a direct damage of the vascular endothelium by aCL.
Lupus 1995 Apr
PMID:Association of anticardiolipin antibodies and abnormal nailfold capillaroscopy in patients with systemic lupus erythematosus. 779 18

To explore the possibility of an interaction between platelets and antiphospholipid antibodies and its relationship with thromboembolic events, platelets from six patients with IgG anticardiolipin antibodies, lupus anticoagulant and thrombosis were isolated by gel filtration. Five patients had primary antiphospholipid syndrome and one had a form secondary to systemic lupus erythematosus. Two patients had mild thrombocytopenia. The six platelet membrane eluates contained less than 1.09 GPL units of anticardiolipin antibodies and displayed no lupus anticoagulant activity. Similarly, lysates of disrupted platelets did not show anticardiolipin or lupus anticoagulant activity. These results suggest that antiphospholipid antibodies do not interact with circulating platelets, at least in patients on oral anticoagulant treatment with no evidence of acute thrombosis.
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PMID:Antiphospholipid antibodies are not present in the membrane of gel-filtered platelets of patients with IgG anticardiolipin antibodies, lupus anticoagulant and thrombosis. 832 68

A 37-year-old woman in acute right heart failure had experienced systemic venous thromboses for 17 years, five miscarriages and repeated pulmonary emboli. For the last 7 years she had been treated symptomatically for pulmonary hypertension. The platelet count was 62,000/microliters, thromboplastin time under phenprocoumon was 22%, partial thromboplastin time was 72 s. Despite anticoagulation with phenprocoumon and heparin (7,500 IU two times daily subcutaneously) new pulmonary emboli occurred and platelet count fell to 12,000/microliters. An increased titre for anticardiolipin antibodies (IgG > 320 GPL U/l, IgM 8 MPL U/l), antinuclear (1:640) and anti-ds-DNA antibodies (> 200 IU/ml) with simultaneous complement consumption suggested secondary antiphospholipid syndrome associated with lupus erythematodes. Treatment with prednisolone (150 mg/d), immunoglobulins (20 mg/d intravenously for 5 days) and heparin (25,000 IU/24 h intravenously) achieved an increase in platelet count to 200,000/microliters within 10 days, but fell again when the prednisolone dose was reduced, recovering under azathioprine, 150 mg/d. Four weeks later the patient died of renewed acute right heart failure.
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PMID:[Pulmonary thromboembolism in antiphospholipid syndrome]. 851 8

Antiphospholipid antibodies (aPL) have been associated with a variety of neurological disorders, mostly linked to focal neuroparenchymal ischemia or infarction. Cerebral ischemia associated with the antiphospholipid syndrome (APS) occurs at a younger age than typical atherothrombotic cerebrovascular disease, is often recurrent, and high positive GPL values are usually linked to the presence of a lupus anticoagulant. When other features of the syndrome are not present and cerebral ischemia occurs only associated with anticardiolipin immunoreactivity, there appears to be no discerning features of these patients unless GPL > 40 for which recurrent thrombo-occlusive events appear to occur more frequently. Other neurological manifestations associated with aPL include cerebral venous sinus thrombosis, ocular ischemia, dementia, including ischemic encephalopathy, and chorea. The role of aPL in migrainous events is controversial and may not play a role in recent, large case-controlled studies. Most seizures in patients harboring aPL are associated with focal brain infarction.
Lupus 1996 Oct
PMID:Neurological aspects of antiphospholipid antibody syndrome. 890 59

41 subjects with highly elevated IgG anticardiolipin antibody (aCL) titers (> 30 GPL-U/ml) were retrospectively evaluated regarding underlying disease, clinical symptoms, and in particular the influence of drugs on aCL titer and clinical symptoms. Whereas 31/41 (76%) fulfilled the criteria for an antiphospholipid antibody syndrome (APS), 10 (24%) did not. About half (47%) of the patients had an autoimmune disease (mainly systemic lupus erythematosus). 26 (63%) had had recurrent thrombophilic events, 3 (7%) recurrent spontaneous abortions and 10 (24%) associated thrombocytopenia. 8/34 (23%) subjects followed up over 6-42 months had a thrombophilic complication again. 6/16 (37%) developed deep venous thrombosis in spite of oral anticoagulation (INR 1.5-3.0), one of three under acetylsalicylic acid treatment and only one subject without therapy. ACL titer decreased (> 12 U/ml) in 20/34 subjects (59%) during the follow-up period, mainly in patients under immunosuppressive treatment due to the underlying autoimmune disease. In comparison with 5/14 subjects (36%) with consistently high aCL, only 3/20 (15%) with decreasing aCL developed thrombosis. Patients treated by immunosuppressive agents had a higher incidence of decreasing aCL than those without. This investigation indicates that a high titer of aCL in asymptomatic subjects does not justify prophylactic anticoagulation therapy. However, if a history of recurrent deep venous thromboses or pulmonary embolisms is established, long-term anticoagulation therapy should be maintained at or above the international normalized ratio (INR) of 3. ACL titers in subjects with an autoimmune disease may decline, as a result of immunosuppressive treatment or otherwise, and this decline is associated with a lower incidence of thrombophilic disorders.
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PMID:[Antiphospholipid antibodies syndrome: follow-up of patients with a high antiphospholipid antibodies titer]. 899 2


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