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)

Lupus anticoagulants and anticardiolipin antibodies are antiphospholipid antibodies (APLAb) with related antigenic specificities and are newly recognized markers for an increased risk of thrombosis. We studied 48 patients who presented with cerebral or visual dysfunction associated with APLAb to help clarify the diagnostic, clinical, laboratory, radiologic, and pathologic features in these patients. Most patients presented with transient cerebral ischemia or cerebral infarction. Recurrent and stereotypic events were frequent. Visual disturbances resulted from amaurosis fugax, retinal arterial or venous occlusion, occipital ischemia, diplopia, and migraine-like disturbances. Three patients presented with severe atypical classic migraine. Recurrent infarcts of brain and eye were significantly associated with the presence of cigarette smoking, hyperlipidemia, and a positive antinuclear antibody. During 44.4 patient-years of prospective follow-up, the combined stroke and systemic thrombotic event rate was 0.27 events per patient-year and was 0.54 events per patient-year if TIA and death were included. Forty (83%) of the patients did not have systemic lupus erythematosus (SLE). Thrombocytopenia was present in 15 (31%) and a false-positive VDRL in 11 (23%) of the patients. Cerebral angiography was normal or revealed large-vessel occlusion or stenosis without changes suggestive of vasculitis. Patients with only transient dysfunction generally had normal radiologic studies, including angiography. Organs and arterial vessels studied pathologically revealed thrombotic occlusive disease without vasculitis. APLAb are strongly associated with an immune-mediated thrombotic tendency, generally in the absence of SLE. Other stroke risk factors may add to the risk of recurrent ischemic events in patients with APLAb.
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PMID:Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. 238 25

We reviewed the clinical and laboratory data of 128 patients with cerebrovascular disease and antiphospholipid antibodies. Cases were evenly divided between men and women, and the mean age of the study group was 46 years. Cerebral infarction occurred in 97 patients, and transient hemispheric ischemic attacks without stroke were recorded in 19; 12 suffered ocular ischemia. Systemic lupus erythematosus was diagnosed in 16% of all cases. Histories of systemic thromboembolic events and recurrent miscarriages were noted in 14% of the patients and in 19% of the women, respectively. Evidence of cerebral infarction preceding the index event was present in 30% of cases. During a mean follow-up of 16 months, nine of 96 (9%) patients sustained new cerebral infarctions. Of 72 echocardiographic studies, 16 (22%) showed valvular abnormalities. Cerebral angiography detected intracranial lesions in 24 of 49 patients (49%). These data indicate that antiphospholipid antibodies can be identified in stroke patients without known autoimmune disorders. They also suggest that antiphospholipid antibody-associated cerebrovascular ischemia may be recurrent and often occurs in patients with systemic thromboembolic events. Our findings should help design a prospective clinical trial that will assess the risk of recurrent thromboembolism in this population, identify stroke risk factors, and address therapy.
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PMID:Clinical and laboratory findings in patients with antiphospholipid antibodies and cerebral ischemia. The Antiphospholipid Antibodies in Stroke Study Group. 200 15

The authors observed three cases (6 eyes) of vaso-occlusive retinopathy associated with the lupus anticoagulant and the related antiphospholipid antibody anticardiolipin. The disease occurred in patients who had no definable autoimmune disease such as systemic lupus erythematosus (SLE) and was characterized by severe bilateral retinal vascular occlusion. There was profound visual loss from intraretinal ischemia as well as vitreous hemorrhage from preretinal neovascularization. Results of laboratory testing showed a prolonged partial thromboplastin time (PTT) in two patients, and the presence of the lupus anticoagulant in all. Treatment with panretinal photocoagulation appeared to stabilize the neovascularization. The role of systemic anticoagulation and immunosuppressive therapy is uncertain.
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PMID:Vaso-occlusive retinopathy associated with antiphospholipid antibodies (lupus anticoagulant retinopathy). 232

We studied 23 patients suffering cerebral ischemia who also had laboratory evidence of either a lupus anticoagulant (LA) or an abnormal anticardiolipin antibody (ACA). Four patients had lupus or a lupus-like illness, three had drug-induced lupus, and 16 had no overt evidence of collagen-vascular disease. Cerebral ischemic events were multiple in 71% of the patients; two patients presented with multi-infarct dementia. Recognized cerebrovascular disease risk factors were present in 57% of the patients. The partial thromboplastin time was prolonged in only 35% of the patients. An LA was identified in 15 of 21 patients tested, and an elevated ACA titer was identified in 10 of 12 patients tested. Simultaneous assays for LA and ACA were discordant in eight of 10 patients tested. LA- and ACA-associated brain ischemia is often recurrent, but other risk factors for cerebrovascular disease are often present. The laboratory findings in such patients may display considerable heterogeneity.
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PMID:Lupus anticoagulants, anticardiolipin antibodies, and cerebral ischemia. 249 72

Lupus-like anticoagulants (LLA), lupus anticoagulant and/or anticardiolipin antibody, are increasingly recognized in association with venous and arterial thrombotic events. We recently reviewed our experience with patients undergoing revascularization for lower-limb ischemia who were found to have LLA. Nine patients had LLA based on a prolongation of the partial thromboplastin time or by anticardiolipin assay by an enzyme-linked immunosorbent assay system. The ages of the patients ranged from 23 to 57 years. There were seven (78%) men, six (67%) blacks, two (22%) diabetic patients, and three (33%) hypertensive patients. One patient had systemic lupus erythematosus. All patients except one were cigarette smokers. Four patients had concurrent regulatory protein abnormalities: three protein C deficiencies, one protein S deficiency, and one plasminogen deficiency. The nine patients had 10 lower-extremity arterial reconstructions with two postoperative failures within 30 days. Patients were anticoagulated with heparin or aspirin after all but one operation. Patients at risk were identified on the basis of age (less than 51 years), unexplained early graft thrombosis, or history of venous or arterial thrombotic events. This group of patients is believed to be at risk for early postoperative thrombosis. Postoperative anticoagulation after revascularization for patients with LLA may be beneficial.
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PMID:Lupus-like anticoagulants and lower extremity arterial occlusive disease. 250 7

The case of a woman with systemic lupus erythematosus with unusual clinical, cutaneous and biologic features and lupus anticoagulant is presented. According to the literature this association is not fortuitous: a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies has been recognized. The cutaneous symptoms of this syndrome include: leg ulcers, livedo reticularis, widespread cutaneous necrosis and distal cutaneous ischemia. In our patient a nearly complete picture of the clinical and biologic features of this syndrome, including a characteristic retinal vein thrombosis is present.
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PMID:[Clinical manifestations associated with the presence of lupus anticoagulant]. 250 30

The presence of a lupus anticoagulant (LA) is paradoxically associated with a high incidence of arterial and venous thrombosis. In a patient with a lupus-like systemic disease, having received phenindione for 11 years, LA was discovered in association with recurrent deep venous thrombosis, a right atrial thrombus, coronary occlusion, arterial hypertension, thrombopenia, and anticardiolipin antibodies without anti-DNA antibodies. Renal cortical ischemia was detected by a tomographic scan. Renal biopsy showed glomerular ischemia and diffuse interstitial fibrosis. After a one-year anticoagulant and steroid therapy, LA has disappeared despite a high level of anticardiolipin antibodies, and renal function remains normal.
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PMID:[Renal cortex ischemia, right atrial thrombosis and coronary occlusion in anti-phospholipid antibody syndrome]. 251 17

This study prospectively evaluates hypercoagulable states in patients under 51 years of age undergoing lower extremity revascularization for ischemia and assesses early outcome after operation. Twenty patients whose ages range from 23 to 50 years (mean 40.8 years) were identified prospectively who underwent lower extremity revascularization and evaluation of hypercoagulability. Fifteen patients were male (75%), 10 were black (50%), six had hypertension (30%), and four were diabetic (20%). All but two were cigarette smokers (90%). Seven aortoiliac procedures and 13 infrainguinal procedures were performed. Six patients had one or more abnormalities of regulatory proteins (protein S deficiency, four; protein C deficiency, three; presence of lupus-like anticoagulant, three; plasminogen deficiency, two). Eight of 17 patients in whom platelet aggregation profiles were obtained showed increased reactivity (47%). Only 4 of 17 patients (24%) were normal when tested for all parameters. Arterial or graft thrombosis developed in four of the 20 patients within 30 days after operation. Hypercoagulability was found in all four patients whose revascularizations failed. A high incidence of hypercoagulable states was found in patients under 51 years of age with lower limb ischemia requiring revascularization. Hypercoagulability may have contributed to early postoperative thrombosis of the vascular procedure.
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PMID:Hypercoagulable states and lower limb ischemia in young adults. 252 8

Intermittent claudication and limb-threatening ischemia caused by occlusion of large and medium-sized arteries are rare manifestations of systemic lupus erythematosus. So far only eight documented cases have been reported, predominantly in young women. In this report two more patients are described. There is no common opinion concerning pathophysiologic mechanism, but immunologic endothelial damage and circulating antibodies to phospholipid probably play a role. The results of medical and surgical treatment are disappointing, and amputation becomes necessary in more than half of the patients.
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PMID:Intermittent claudication and limb-threatening ischemia in systemic lupus erythematosus and in SLE-like disease: a report of two cases and review of the literature. 266 62

In the case we have reported, there is a combination of factors that can be attributed to SLE and its associated complications, which may explain the transient hypodensities seen on cranial CT scan. Vasculitis and uncontrolled hypertension lead to arterial spasm, ischemia, and increased vascular permeability, all of which eventually cause cerebral edema. This edema may manifest itself clinically as profound but transient neurologic deficits.
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PMID:Reversible profound neurologic deficits associated with transient cranial CT abnormalities and systemic lupus erythematosus. 271 92


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