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

Cerebral infarction is the most common arterial thromboembolic complication in the anti-phospholipid antibodies (aPL) syndrome. In an effort to clarify the roles of aPL in the pathogenesis of cerebral infarction in patients with SLE, we examined the levels of anti-cardiolipin/2-glycoprotein I antibodies (anti-CL/beta2-GPI) and anti-phosphatidylserine/prothrombin anti-bodies (anti-PS/PT) in addition to lupus anticoagulant (LA) activity in 126 patients with SLE (35 with cerebral infarction and 91 without thrombosis). Both anti-CL/beta2-GPI and anti-PS/PT strongly correlated with the presence of LA activity. The prevalence of cerebral infarction was obviously higher in the patients who had both anti-CL/beta2-GPI and anti-PS/PT (76.5% [26/34 cases], p<0.0001) than in the other patients having anti-CL/beta2-GPI or anti-PS/PT alone or neither of them (9.8% [9/92 cases]). Furthermore, we studied the in vitro effects of anti-CL/beta2-GPI and/or anti-PS/PT on the enhancement of platelet activation induced by stimulation with a low concentration of adenosine diphosphate (ADP). The purified IgG containing both anti-CL/beta2-GPI and anti-PS/PT caused significant enhancement of platelet activation caused by ADP. However, the purified IgG containing either anti-CL/beta2-GPI or anti-PS/PT had no enhancing effects on it. Furthermore, platelet activation was generated by the mixture of anti-CL/beta2-GPI-IgG and anti-PS/PT-IgG prepared from individual patients, but not by each fraction alone. These results indicate that anti-CL/beta2-GPI and anti-PS/PT may cooperate to promote platelet activation, which may contribute to the risk of cerebral infarction in patients with SLE.
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PMID:Strong correlation between the prevalence of cerebral infarction and the presence of anti-cardiolipin/beta2-glycoprotein I and anti-phosphatidylserine/prothrombin antibodies--Co-existence of these antibodies enhances ADP-induced platelet activation in vitro. 1511 58

A 52-year-old previously healthy woman was admitted to our hospital for status epilepticus in November 1999. She had not taken oral contraceptives. After treatment with intravenous diazepam and phenytoin, she did not develop seizures anymore. When she became alert, there was a mild left hemiparesis. Lumbar puncture showed an opening pressure of 145 mm H2O, and the cerebrospinal fluid was acellular. Cranial MR imaging demonstrated thrombosis of the superior sagittal sinus and fresh infarction in the right frontal lobe. Plasma fibrinogen, fibrin degradation product, and prothrombin fragment 1 + 2 levels were elevated. Proteins S and C activities and anti-thrombin III levels were within the normal range. Lupus anticoagulant and anti-cardiolipin antibody were negative. She was treated with continuous heparin infusion for ten days and with oral warfarin thereafter. Six months after the first admission, platelet count became more than 400 x 10(3)/microliter. In July 2002, she developed slowly progressive monoplegia of the left arm. Cranial MR imaging demonstrated patent superior sagittal sinus, fresh infarction in the right parietal lobe, and old small infarction in the right corona radiata. The patient was maintained on warfarin and 100 mg of aspirin thereafter. In September 2002, platelet count was 737 x 10(3)/microliter. Bone marrow examination showed increased megakaryopoiesis with normal erythroid and myeloid series and no chromosomal aberrations. Serum C-reactive protein and iron levels were in the normal range. An abdominal ultrasound demonstrated mild splenomegaly. Thus, we made a diagnosis of essential thrombocythemia (ET). ET causes thrombotic events in the course of the disease at a rate of 7% per year. Cerebral infarction is not uncommon, but occurrence of cerebral sinus thrombosis has been rarely reported. Recently, several cases have been reported in which cerebral infarction was the first manifestation of ET even with platelet counts lower than 600 x 10(3)/microliter. To our knowledge, there have been no reported cases of ET presenting with cerebral venous sinus thrombosis. Platelet count should be monitored in the patients with venous sinus thrombosis of undetermined etiology.
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PMID:[Superior sagittal sinus thrombosis as first manifestation of essential thrombocythemia]. 1519 36

A 32-year old woman with antiphospholipid antibody syndrome (APS) developed severe thrombocytopenia, elevated liver enzymes and progressive cerebral thrombosis a few days after preterm delivery by caesarean section. Her condition deteriorated despite treatment with low dose aspirin, anticoagulation by heparin and iv glucocorticoid administration. Intravenous immunoglobulin (IVIG) on three consecutive days was followed by rapid resolution of her neurological impairment and increasing platelets counts. The temporal association between IVIG and reversal of both neurological impairment and platelet number strongly indicates a specific effect of IVIG administration in this condition. It is proposed that IVIG therapy is considered as a therapeutic option in APS patients with progressive cerebral infarction despite optimal use of anticoagulant and immunomodulating agents.
Lupus 2004
PMID:IVIG treatment for progressive stroke in the primary antiphospholipid antibody syndrome. 1593 44

A 41-year-old woman who had suffered from systemic lupus erythematosus (SLE) for 22 years presented with signs of neurological deficits. CT-scanning of the brain revealed hypodense lesions that suggested cerebral infarction due to vasculitis in SLE. However, in spite of intensified immunosuppressive therapy, she showed rapid neurological deterioration. After extensive, additional examinations and tests, the diagnosis was finally changed to progressive multifocal leukoencephalopathy, caused by an opportunistic infection by the JC polyomavirus. Neurological and psychiatric symptoms frequently occur in patients with SLE. The differential diagnosis of these symptoms in SLE is extensive and includes, on the one hand, primary neurological and psychiatric diseases related to direct involvement of the nervous system by SLE, and on the other hand, secondary syndromes arising as a result of complications of the SLE or the immunosuppressive treatment. Opportunistic infections are often an important secondary cause of neurological and psychiatric syndromes in patients with SLE. The clinical symptoms and radiological cerebral signs are non-specific and usually do not suffice to differentiate between the various syndromes. Since each syndrome requires its own specific clinical approach and treatment, extensive diagnostics are mandatory before the diagnosis 'cerebral lupus' can be made and immunosuppressive therapy can be started or intensified.
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PMID:[Progressive multifocal leukoencephalopathy in a patient following long-term immunosuppressive therapy for systemic lupus erythematosus]. 1652 99

A 39-years-old woman was admitted to our hospital with musculoskeletal complaints (myalgias and symmetric arthralgias in proximal interphalangeal, metacarpophalangeal joints of the hands and in knees), systemic symptoms like fever, fatigue, malaise and a six months previous history of a transient ischemic attack. The presence of antibodies to double-stranded deoxyribonucleic-acid (DNA) and antiphospholipid antibodies led to the diagnosis of systemic lupus erythematosus with secondary antiphospholipid syndrome. Cerebral infarction develops significantly more often in patients with lupus and antiphospholipid antibodies, but other clinical syndromes are associated with lupus anticoagulant: cognitive dysfunction, seizures, polyneuropathy, aseptic meningitis, myelopathy.
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PMID:[Systemic lupus erythematosus with neurologic onset and secondary antiphospholipid syndrome. A Case Study]. 1660 81

We investigated positive rate of lupus anticoagulant (LA) according to the each understanding disease in our hospital. 596 cases (F/M 477/149, 7-87 y.o.) were examined from 2003 to 2004 years. LA tests were performed using 2 methods such as kaolin clotting time (KCT) mixing test and dilute Russell's viper venom time (dRVVT). The LA tests were most frequently ordered in dermatology, and the most common purpose of LA test was the check of existence of antiphospholipid (aPL) in patients with collagen diseases. The LA positive rate was the highest in patients with SLE among the collagen diseases, and in patients with cerebral infarction among the thrombotic diseases. The LA positive rate exceeded 40% in ITP and livedo reticularis. Moreover, LA positive rate was 16% in preoperative tests of the orthopedic patients without any physical diseases. Thus, it was suggested that there were considerable numbers of the asymptomatic LA positive persons. The LA positive cases based on KCT only accounted for about 60% of all the LA positive cases. Among the thrombotic patients, there were not the DVT/PE patients with only KCT positive. On the other hands, the KCT positive rate was higher than the dRVVT positive rate in patients with cerebral infarction. There were not dRVVT single positive cases in patients with recurrent abortion and ITP, but KCT single positive case accounted for about 90%. From these results, it is suggested that there is a difference in KCT and dRVVT about detecting aPL, and that care should be taken to interpret the LA test.
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PMID:[The positive rate of lupus anticoagulant according to the each understanding disease in our hospital]. 1706 71

This review focuses on the several coagulation disorders (the so called hypercoagulable states) that are associated with cerebral venous thrombosis. Hypercoagulable states likely explain the high percentage of cases of cryptogenic cerebral infarction in young people. The most common of the hereditary defects appear to be deficiency of antithrombin III, protein C or protein S, activated protein C resistance and prothrombin 20211A mutation. In a large majority of cases activated protein C resistance is due to the presence of factor V Leiden. Antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) represent an acquired disorder of coagulation. Rare defects include heparin cofactor II (HC II), plasminogen or tissue plasminogen activator deficiency (TPA), elevated plasminogen activator inhibitor-1 (PAI-1) and dysfibrinogenemia. Hyperhomocystinemia is responsible for both arterial and venous thrombosis. A work-up to identify one of the recognizable hypercoagulable states is indicated, especially in younger patients with stroke. Laboratory evaluation for hypercoagulable states may also often be indicated in those patients who do not have other obvious risk factors for their stroke. If from clinical history, family history and/or laboratory studies, a patient is felt to have a hypercoagulable state, the decision for long term chronic anticoagulation needs to be individualized. If a hereditary hypercoagulable state is found, it also may be appropriate to recommend screening of other family members.
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PMID:Haematologic disorders and cerebral venous thrombosis. 1718 75

We describe a 58-year old female patient with rapid development of arterial and venous thromboembolisms, including deep vein thrombosis (DVT) in the lower limbs, recurrent cerebral infarctions and bilateral pulmonary emboli. Her laboratory data on admission showed positive anticardiolipin antibody of IgG isotype (IgG aCL) and positive anti-beta2 glycoprotein-I antibody of IgG isotype (IgG abeta2-GPI), and decreased protein C activity and protein S antigen. Systemic examinations revealed the presence of an ovarian cancer. Surgical resection was attempted, but her cancer infiltrated the pelvic wall and could not be resected. Despite treatment with unfractionated heparin followed by warfarin, she died due to recurrent episodes of cerebral infarction. This case was considered as probable catastrophic antiphospholipid syndrome (CAPS), which might be associated with ovarian cancer. Known as Trousseau's syndrome, arterial and, more commonly, venous thrombosis is a frequent complication of cancer and sometimes a harbinger of occult cancer. Our case indicates that there is an overlap between antiphospholipid syndrome (APS) and Trousseau's syndrome. It is important to bear in mind that a thrombotic event associated with cancer can be the first manifestation of CAPS.
Lupus 2007
PMID:Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature). 1728 88

In antiphospholipid syndrome (APS), there is a high prevalence of valvular heart disease which leads to increased risk of thrombo-embolic events, in particular, cerebrovascular events. We present a patient with cerebral infarction, previous deep-vein thrombosis, and miscarriages with positive lupus anticoagulant and anticardiolipin antibodies. Echocardiographic examination revealed mitral valve leaflet thickening and verrucous vegetations consistent with Libman-Sacks endocarditis, which is commonly associated with APS. In patients with combined Libman-Sacks endocarditis and antiphospholipid antibodies, anticoagulation therapy with warfarin is indicated due to high risk of valvular thrombus formation and subsequent embolization.
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PMID:Libman-Sacks endocarditis and cerebral embolization in antiphospholipid syndrome. 1826 21

Systemic lupus erythromatosus is often associated with an antiphospholipid syndrome (APS). A high prevalence of valvular heart disease in APS leads to increased risk of embolic events, particularly cerebrovascular. We present a patient with cerebral infarction, with positive lupus anticoagulant, anticardiolipin antibodies and factor V Leiden mutation. Echocardiographic examination revealed mitral valve anterior leaflet thickening and verrucous vegetations consistent with Libman-Sacks endocarditis, which is commonly associated with APS.
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PMID:[Recurrent multi-focal cerebral stroke complicating Libman-Sacks endocarditis in a young woman with systemic lupus erythromatosus]. 1925 90


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