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)

Primary antiphospholipid syndrome or PAPS is characterised by antiphospholipid antibodies and arterial and/or venous thromboses. Numerous other clinical features have been shown to be related to this syndrome. Chorea is a well known but rare phenomenon in systemic lupus erythematosus; it has been shown to be strongly related to the presence of antiphospholipid antibodies. We describe two patients with chorea that appeared to be caused by the PAPS.
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PMID:[Chorea and primary antiphospholipid syndrome]. 140 67

Primary antiphospholipid syndrome (PAPS) is considered a distinct entity from SLE and patients with PAPS are generally regarded as being dsDNA antibody negative. Levels of IgG and IgM ss and ds DNA antibodies were measured by ELISA in 30 patients who fulfilled the criteria for the diagnosis of PAPS. We compared these patients with 20 normal controls and seven patients with idiopathic SLE. We also examined all the sera for anti-nuclear antibodies by Hep-2 cells and for dsDNA antibodies by Crithidia. We found that 16 patients with PAPS had antibodies to ss and/or dsDNA. Only three of the 16 positive patients had both IgG and IgM anti-DNA antibodies. Twelve patients had anti-nuclear antibodies, but only two were weakly positive for dsDNA antibodies by Crithidia immunofluorescence. Eleven out of 30 patients with PAPS had IgM anti-dsDNA antibodies compared to two out of the seven SLE patients. The PAPS patients with anti-DNA antibodies were clinically indistinguishable from the PAPS patients without antibodies against DNA. Our results show that 53% of patients with PAPS had antibodies to DNA which supports the view that PAPS and SLE are probably overlapping disorders.
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PMID:Anti-DNA antibodies in the primary antiphospholipid syndrome (PAPS) 822 Sep 29

Primary antiphospholipid syndrome (pAPS) can be experimentally induced in mice by immunization with anti-cardiolipin (aCL) antibodies (Abs). Recently, we have pointed to the pathogenic role of antiphosphatidylserine (aPS) Abs by inducing an experimental model of pAPS in naive mice following passive transfer of human aPS to the tail vein of ICR mice. The aim of the present study was to induce experimental pAPS in mice following active immunization with aPS. Mice were immunized with IgG and IgM aPS, purified from two patients with pAPS. The sera of the mice were examined for the presence of different antiphospholipid Abs, and the beta 2 GPI dependency of aPS, using an enzyme-linked immunosorbent assay (ELISA). Inhibition ELISA studies, with silica beads coated with phospholipids, were used to detect cross-reactivity of aPS or aCL to other phospholipids. The mice were also tested for the presence of thrombocytopenia, prolonged activated partial thromboplastin time (APTT), and the percentage of fetal resorptions. The purified IgG aPS but not IgM aPS had a strong lupus anticoagulant activity. Both IgG and IgM aPS were beta 2 GPI dependent and did not bind PS in the absence of the glycoprotein. The mice immunized with IgG aPS, but not IgM aPS, developed high titers of mouse aPS. The mice generated polyspecific Abs, cross-reacting with aCL and aPS, as well as individual aPS or aCL Abs. Only the mice immunized with IgG aPS developed clinical parameters of pAPS: prolonged APTT, thrombocytopenia, and an increased fetal resorption rate. In conclusion, active immunization with IgG, but not IgM, aPS induces experimental pAPS in naive mice, thus pointing to the participation of aPS in the idiotypic network.
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PMID:The pathogenic role of anti-phosphatidylserine antibodies: active immunization with the antibodies leads to the induction of antiphospholipid syndrome. 859 79

A 36-year-old female with a history of recurrent pregnancy loss experienced sudden onset of disturbance in consciousness, with right hemiparesis and total aphasia. Computed tomography revealed a massive hemorrhage in the left frontal lobe, and angiography showed occlusion of the anterior two-thirds of the superior sagittal sinus. Laboratory investigations detected the presence of lupus anticoagulant, elevation of the anticardiolipin beta 2-glycoprotein I complex antibody level, and a decreased protein S activity level. There were no underlying conditions, such as connective tissue disorders, malignancies, infectious diseases, and drug-induced disorders, so the diagnosis was primary antiphospholipid syndrome. Primary antiphospholipid syndrome should be considered in the evaluation of patients with "idiopathic" or "primary" sinus and cerebral venous thrombosis.
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PMID:Superior sagittal sinus thrombosis associated with primary antiphospholipid syndrome--case report. 954 Mar 31

Three patients, aged five to 16 years, developed chorea as the only or main clinical manifestation of primary antiphospholipid syndrome. In two cases, complaints were self-limited five to eight months after onset. In one patient, the clinical course was complicated by valvulitis. Under corticosteroid treatment, chorea disappeared and cardiac involvement stabilised. Primary antiphospholipid syndrome is a probably under-recognised differential diagnosis of choreatic syndromes in childhood. Assessment of anticardiolipin antibodies and/or lupus anticoagulant should be an obligatory part of the diagnostic work-up of such patients. Early diagnosis of primary antiphospholipid syndrome may improve clinical management and prognosis.
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PMID:Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood. 1083 88

Primary antiphospholipid syndrome is associated with an increased risk of vascular thrombosis. The authors describe a young patient without any risk factor for coronary artery disease who was admitted to the hospital because of a transient cerebral ischemic attack. Standard EKG showed signs of a previous silent inferior wall myocardial infarction, confirmed by echocardiography, technetium-99 scintigraphy, and left ventricular angiography. Coronary arteries appeared normal at angiography. Blood tests showed the presence of antiphospholipid antibodies and lupus anticoagulant. Since there is evidence that these antibodies are associated with an increased risk of microvascular thrombosis, the authors conclude that this silent myocardial infarction could be caused by a cardiac microvascular disease accompanying the antiphospholipid syndrome.
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PMID:Myocardial infarction with normal coronary arteries in a patient with primary antiphospholipid syndrome--case report and literature review. 1171 32

Primary antiphospholipid syndrome (APS) is a disease producing vascular thrombus with antiphospholipid antibody without association with autoimmune diseases as systemic lupus erythematosus. Retinal vein occlusion is a rare vascular manifestation in primary APS. We describe 2 cases of primary APS presenting with developing blurred vision. Each had central retinal vein occlusion and high titer of IgG anticardiolipin antibody.
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PMID:Retinal vein occlusion in two patients with primary antiphospholipid syndrome. 1185 60

Primary antiphospholipid syndrome (APS) is associated with arterial and venous thrombosis. However, a small number of patients present with visceral aneurysms. Although such aneurysms are rare, their presence in patients who are usually treated with lifelong anticoagulation raises important therapeutic problems, in view of the risk of aneurysm rupture and acute abdominal hemorrhage. We report the case of a young woman with APS who presented with abdominal bleeding due to ruptured common hepatic artery aneurysm. She was successfully treated by proximal ligation. The features of such aneurysms are discussed.
Lupus 2007
PMID:Hepatic artery aneurysm rupture in a woman with primary antiphospholipid syndrome. 1757 38

Primary antiphospholipid syndrome (APS) is characterized by venous or arterial thrombotic events and/or recurrent abortions, fetal death, preeclasmpsia, eclampsia in the presence of anticardiolipin antibodies or lupus anticoagulant, in the absence of accompanying diseases. Antiphospholipid antibodies can activate endothelial cells, and were recently implicated in atherosclerosis. To assess potential endothelial impairment and early signs of atherosclerosis, flow-mediated (endothelium-dependent) and nitrate-mediated (endothelium independent) vasodilation, as well as von Willebrand factor antigen level and carotid artery intima-media thickness (IMT) were measured in patients with primary antiphospholipid syndrome and in healthy controls. Flow-mediated vasodilation in patients with primary APS was significantly lower than that of controls (3.43 +/- 2.86% versus 7.96 +/- 3.57%; P < 0.0001). We also found significantly higher von Willebrand antigen levels in patients with primary APS than in the control group (157.91 +/- 52.45% versus 125.87 +/- 32.8%; P = 0.012). Moreover, carotid artery IMT was significantly larger in the primary APS group compared to controls (0.714 +/- 0.2 mm versus 0.58 +/- 0.085 mm; P = 0.0037). Our results reflect ongoing endothelial damage and accelerated atherosclerosis in patients with primary APS, and suggest that vasoprotective therapy may be beneficial in the treatment of these patients.
Lupus 2007
PMID:Impaired endothelial function and increased carotid intima-media thickness in association with elevated von Willebrand antigen level in primary antiphospholipid syndrome. 1767 Aug 48

Primary antiphospholipid syndrome (APS) is a well-defined entity characterized by spontaneous and recurrent abortion, thrombocytopenia and recurrent vascular thromboses (arterial and venous). Left ventricular thrombus mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. In this report we describe a 39 year-old man admitted to hospital presenting with left hemiparesis and a peripheral embolism. He had no history of thrombotic events. Transthoracic echocardiography showed a large, polypoid and mobile mass (4.0 x 1.2 cm) attached to the apex of the left ventricle, highly suggestive of primary cardiac tumor. The patient subsequently underwent open heart surgery. The histological examination showed an older thrombus and a fresh thrombus. Post-operative laboratory tests showed lupus anticoagulant activity, confirming the primary APS diagnosis. The patient initiated treatment with oral anticoagulation (INR levels between 2 and 3) and was discharged 29 days after surgery. At ten month follow-up, he was symptom-free with long-term anticoagulation therapy. No evidence of intracardiac mass recurrence on two-dimensional echocardiography was seen. Intracardiac thrombus has been rarely reported as a complication of primary APS. Left ventricular mass mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. Pre-operative investigations could not distinguish such a thrombus from a cardiac tumor and the diagnosis was made post-operatively.
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PMID:Left ventricular thrombus mimicking primary cardiac tumor in a patient with primary antiphospholipid syndrome and recurrent systemic embolism. 1995 93


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