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

A 23-year-old woman with SC hemoglobinopathy and systemic lupus erythematosus had recurrent amaurosis fugax in her right eye. Extraordinary retinal vascular events were observed and photographed during the amaurotic attacks. Prednisone therapy halted the attacks. We believe a focal lupus vasculopathy involving the central retinal artery caused the amaurosis fugax.
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PMID:Amaurosis fugax associated with SC hemoglobinopathy and lupus erythematosus. 43 86

Eight patients, 3 with systemic lupus erythematosus (SLE) or "SLE-like" disease, 1 with sarcoidosis, and 4 with no connective tissue disease had transient ischemic attacks (TIA) or cerebral infarctions associated with high levels of anticardiolipin antibodies (ACA). Cerebral ischemic events included amaurosis fugax, recurrent hemispheric TIA, cerebral infarction, and multi-infarction dementia. Treatment with acetylsalicylic acid was ineffective in 3 patients. Warfarin, alone or in combination with dipyridamole or steroids, may reduce the risk of further cerebrovascular events.
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PMID:Cerebral ischemia associated with anticardiolipin antibodies. 164 13

For several months a 23-year-old woman had been suffering from increasingly frequent attacks of bilateral amaurosis fugax. They affected only individual segments of a monocular visual field and finally occurred several times daily. Physical and cardiological examinations as well as echocardiography were within normal limits. Laboratory tests revealed a slight increase in erythrocyte sedimentation rate and high titres of serum antibodies against phospholipids. Fundoscopy did not show any vascular changes. Because vasculitis--possibly as an expression of systemic lupus erythematodes--was suspected, high-dosage treatment with steroids was commenced, but failed to influence the visual disorder. While steroid dosage was gradually decreased, administration of acetylsalicylic acid (for three months 100 mg daily, then three times daily 100 mg) brought about complete disappearance of the visual signs. Their cause was probably a reversible platelet aggregation induced by antiphospholipid antibodies which, because of the physiological characteristics of the terminal retinal vascular bed brought about the isolated sign of recurrent amaurosis fugax.
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PMID:[The antiphospholipid syndrome. The neurological complications and the therapeutic possibilities]. 193 73

A woman with a history of polyarthralgias appeared to develop systemic lupus erythematosus and lymphoma simultaneously. The diagnosis of the concurrent lymphoma was made on biopsy of a left axillary lymph node. Generalized lymphadenopathy, splenomegaly and pruritus had given rise to suspicion of an underlying lymphoma. The lymphoma responded well to chemotherapy. Her condition was further complicated by an arterial occlusion involving the right 2nd toe which was eventually amputated, transient ischemic attacks (amaurosis fugax), livedo reticularis and thrombocytopenia which were accompanied by elevations of IgM anticardiolipin antibodies and a biological false test for syphilis. The lupus anticoagulant test was not performed as she was given anticoagulation therapy.
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PMID:Systemic lupus erythematosus and lymphoma: association with antiphospholipid syndrome. 202 23

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

The lupus anticoagulant, an acquired circulating serum gamma-globulin, prolongs all phospholipid-dependent coagulation tests. Recent associations of the lupus anticoagulant and focal cerebral and/or ocular ischemia have been made. We present 5 cases of lupus anticoagulant-associated cerebrovascular ischemia and review all reported cases for the first time. Clinical spectra, cerebral angiographic findings, associated conditions, and response to therapy are presented. Typical features include a relatively young age (mean 39 years), female preponderance, transient ischemic attacks (including amaurosis fugax) or stroke, and normal or large vessel occlusions on angiography. Commonly associated conditions were systemic lupus erythematosus (34%), noncerebral venous thrombosis (31%), hypertension (28%), false-positive VDRL (28%), and spontaneous abortions (22%). Four of our 5 patients (all without systemic lupus erythematosus) and 11 of the 20 (55%) patients in the literature without systemic lupus erythematosus had other definite stroke risk factors coexisting. Response to therapy was highly variable, with no clear beneficial effect of corticosteroids.
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PMID:Cerebrovascular ischemia associated with lupus anticoagulant. 310 Dec 33

One of the clinical hallmarks of antiphospholipid syndrome is the development of neurological complications, namely cerebral ischaemia, chorea, multi-infarct dementia, amaurosis fugax, migraine and transverse myelitis. An animal model should include the development of measurable neurological deficits and evidence of cerebral infarction. Although there are a number of mouse models for fetal loss, there has been no convincing model for the neurological complications of the antiphospholipid syndrome. One explanation for the high frequency of neurological events in antiphospholipid syndrome is a vulnerability of the cerebral vasculature to the hypercoagulable state associated with the syndrome. A greater appreciation of the differences in the regulation of coagulation between the systemic and cerebral vasculatures may be key to understanding the apparent predilection for central nervous system involvement in the antiphospholipid syndrome.
Lupus 1994 Aug
PMID:Models for central nervous system complications of antiphospholipid syndrome. 780 11

The purpose of our study was to define the neuroimaging features of the cardiolipin antibody syndrome. Thirty-eight patients with elevated anticardiolipin antibody titers were studied with magnetic resonance imaging or computed tomography or both. Two patients underwent cerebral angiography. All patients had recurrent transient ischemic attacks, amaurosis, or strokes. One patient had normal imaging findings. The remaining patients had a combination of infarction and atrophy. Focal infarcts, the most common finding, were seen in 32 patients. Cerebral atrophy was seen in 26 patients and was the only radiographic finding in 5. Angiography demonstrated dramatic abnormalities in the distal portions of the anterior and posterior circulations, with multiple stenosis and occlusions and extensive pial and transdural collateral networks. The cardiolipin antibody syndrome should be suspected in young patients with transient ischemic attacks or strokes in the absence of the usual risk factors for cerebrovascular disease. The presence of raised anticardiolipin antibody titers or the cardiolipin antibody syndrome in patients with lupus, in those with other connective tissue diseases, and in patients without overt manifestations of an autoimmune disorder should be viewed as a risk factor for future ischemic cerebrovascular events. Further understanding of the precise role of these antibodies in the pathogenesis of vascular thrombosis may lead to a better understanding of the mechanisms underlying certain forms of stroke.
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PMID:The neuroimaging features of the cardiolipin antibody syndrome. 911 34

The classical clinical picture of the antiphospholipid syndrome (APS) is characterized by venous and arterial thromboses, fetal losses and thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or antibodies to the protein "cofactor" b2 glycoprotein I. Single vessel involvement or multiple vascular occlusions may give rise to a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual and the time interval between them also varies considerably from weeks to months or even years. Deep vein thrombosis, sometimes accompanied by pulmonary embolism, is the most frequently reported manifestation in this syndrome. Cerebrovascular accidents-either stroke or transient ischemic attacks-are the most common arterial thrombotic manifestations. Early and late fetal losses, premature births and pre-eclampsia are the most frequent fetal and obstetric manifestations. Additionally, several other clinical features are relatively common in these patients, i.e., thrombocytopenia, livedo reticularis, heart valve lesions, hemolytic anemia, epilepsy, myocardial infarction, leg ulcers, and amaurosis fugax. However, a large variety of other clinical manifestations have been less frequently described in patients with the APS, with prevalences lower than 5%. These include, among others, large peripheral or aortic artery occlusions, Sneddon's syndrome, chorea, transverse myelopathy, intracardiac thrombus, adult respiratory distress syndrome, renal thrombotic microangiopathy, Addison's syndrome, Budd-Chiari syndrome, nodular regenerative hyperplasia of the liver, avascular necrosis of the bone, cutaneous necrosis or subungual splinter hemorrhages. In this article, some of these "unusual" manifestations are reviewed.
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PMID:Unusual manifestations of the antiphospholipid syndrome. 1279 62

The presence of antiphospholipid (aPL) antibodies and antiphospholipid syndrome (APS) was researched in 57 children and adolescents with systemic lupus erythematosus (SLE). The frequency of aPL antibodies was 75.4% (anticardiolipin 70.2% and lupus anticoagulant 29.1%). The positivity for these antibodies fluctuated during the course of the disease. No association was found between aPL antibodies and clinical or laboratory manifestations or the autoantibodies studied, nor with the activity or gravity of the SLE. APS was diagnosed in 14% of the cases (eight patients), on average three years after the diagnosis of SLE. Four patients had arterial thrombosis (stroke, three; transient ischaemic attack, one; amaurosis fugax, two; renal, one), one presented with deep vein thrombosis (DVT) and three had involvement of small calibre vessels (osteonecrosis, two; transverse myelitis, one). Recurrences were observed in three of the eight cases (37.5%), with a mean interval of 13 months between the events. The presence of APS was associated with haemolytic anaemia, leukopenia, thrombocytopenia, coagulation abnormalities, ischaemic cerebrovascular accidents, amaurosis fugax, osteonecrosis and interstitial pneumonitis. A negative association was observed between APS and the presence of anti-Ro antibodies.
Lupus 2003
PMID:Antiphospholipid antibodies and antiphospholipid syndrome in 57 children and adolescents with systemic lupus erythematosus. 1466 97


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