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

When the diagnosis of antiphospholipid antibody syndrome (aPS) is being considered in persons who have experienced an ischemic stroke or a transient ischemic attack, it is important to gauge how well the history and laboratory data fit with this diagnosis as opposed to other causes of infarct. The fewer the number of typical vascular disease risk factors and the more confirmatory the laboratory findings (ie, high anticardiolipin antibody titers or presence of lupus anticoagulant), the stronger the suspicion of aPS. There are no good prospective randomized data on stroke prevention with any form of therapy following a first stroke or transient ischemic attack associated with antiphospholipid antibody (aPL). Short-term anticoagulation with an International Normalized Ratio (INR) of 2.0 to 3.0 may be considered in these cases, as could antiplatelet agents if no clear cardiac source is found. If anticoagulation is chosen, if there is no recurrence, and if the level of aPL appears to decline, a change to a stroke prevention medication that may carry less risk, such as an antiplatelet agent, may be appropriate. In patients with more typical vascular disease risk factors and less confirmatory laboratory evidence of aPS (ie, low to moderate titer of anticardiolipin antibodies and lack of other clinical or serologic evidence of aPS), a more conservative approach may be considered and antiplatelet therapy initiated. In either situation, close follow-up for recurrent thrombosis and aPL can help determine whether more or less aggressive (risky) therapies should be considered. Results of randomized controlled trials of different treatment options for aPS are awaited.
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PMID:Antiphospholipid Antibody Syndrome. 1109 70

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

We report on the characteristics of 21 patients with hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver, and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all patients. Cells were CD3+CD5-, expressed the gammadelta T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the Vdelta1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died despite consolidative or salvage high-dose therapy. In conclusion, HSgammadeltaTCL is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis, and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most patients.
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PMID:Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. 1290 41

The aim of the study was to assess the relationship between ischemic cerebrovascular accidents (ICVAs), that is, transient ischemic attack (TIA) or stroke, and left-sided heart valve abnormalities (LHVAs) in patients with systemic lupus erythematosus (SLE). In total, 71 consecutive SLE patients were studied. At baseline, history, clinical and laboratory evaluations, as well as trans-thoracic echocardiography (TTE) were performed. From the original population, so patients were followed up for a mean time of 5.80 +/- 1.53 years. After a mean period of 5.39 +/- 1.42 years; 40 patients underwent a repeat TTE. Previous ICVA history was present at baseline in 16 patients (22.5%). Of these, 13 (81.2%) had evidence of LHVAs on TTE. Previous ICVAs were significantly associated to diagnosis of secondary anti-phospholipid syndrome (SAPS), positivity for anti-cardiolipin antibodies (aCl), and LHVAs. Multivariate analysis confirmed the correlation between previous ICVAs and LHVAs. LHVAs were not more commonly observed in patients with SAPS compared to patients without SAPS. At the end of follow-up, irrespective of any differences in antithrombotic treatment, ICVAs had occurred in 13 patients. During follow-up, ICVAs had recurred in seven patients, while a first event TIA occurred in one patient. Multivariate analysis confirmed the relationship between ICVAs and LHVAs, and a trend towards a positive correlation of the former with SAPS. This study demonstrates that LHVAs represent a compelling risk factor for the development of ICVAs in SLE patients. Conversely, SAPS and aCl positivity, although associated with ICVAs, did not clearly correlate with LHVAs in our study. These results provide insight on the pathogenesis of ICVAs and may give clues on the potential efficacy of preventive/therapeutic strategies in different SLE subpopulations.
Lupus 2003
PMID:Left-sided heart valve abnormalities and risk of ischemic cerebrovascular accidents in patients with systemic lupus erythematosus. 1466 95

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

Antiphospholipid syndrome is considered to be a cause of an acquired hypercoagulable state leading to stroke and transient ischemic attack. Antiphospholipid antibodies (aPL) comprise a heterogeneous group of autoantibodies. Among them, lupus anticoagulant (LA) and beta 2-glycoprotein I dependent anticardiolipin antibody (beta 2-GPI aCL) are important and commonly measured. Recently, LA has been considered to be closely related to phosphatidylserine anti-prothrombin antibody. APL is an independent risk factor for first-ever ischemic stroke and a prognostic marker of recurrent stroke. The precipitating factors for the occurrence of stroke are the presence of beta 2-GPI-dependent aCL, a GPL aCL level of more than 40, and the simultaneous presence of lupus anticoagulant. Several mechanisms are believed to be involved in the thrombotic process in patients with antiphospholipid antibodies. Human activated protein C functions as a potent anticoagulant in human plasma by inhibiting the activity of coagulation cofactors Va and VIIIa. Activation of protein C is impaired in patients with aPL. Recently, the presence of aPL has been considered to be contributory factor for the development of atherosclerotic lesions. Transgenic mouse lacking the LDL receptor develop accelerated arteriosclerosis upon immunization with beta 2-GPL Several therapeutic options are available for the prevention of ischemic stroke in patients with aPL, such as antiplatelet, anticoagulant, and immunosuppressive therapy. The rate of recurrence in patients undergoing antiplatelet and anticoagulation combination therapy was found to be lower than that in patients receiving other forms of therapy. The WARSS-APASS collaborative study showed that there was no difference in the recurrence rate between aPL patients receiving antiplatelet or anticoagulation therapy alone. APL has been investigated in other neurological disorders such as multiple sclerosis, chorea, migraine and convulsion. The association of aPL with multiple sclerosis remains debatable. APL could be a contributory factor for the development of convulsion, but not for migraine.
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PMID:[Neurological aspects in antiphospholipid syndrome]. 1515 54

Thirty-seven patients with systemic lupus erythematosus underwent complete clinical and laboratory evaluations, including antiphospholipid antibodies and lupus anticoagulant, magnetic resonance imaging of the brain, and transesophageal echocardiography. Cerebrovascular disease manifested as stroke, transient ischemic attack, or cerebral infarcts in patients with nonfocal neurologic deficits was detected in 19 patients (51%), and significant left-sided valvular heart disease in 25 (68%). Valve vegetations, valve thickening, valve regurgitation, and lupus anticoagulant antibody occurred 2 to 3 times more often in patients with than without cerebrovascular disease (all p < or =0.04) and were the only independent predictors of cerebrovascular disease (odd ratios 5.3 to 10.6, all p < or =0.03). Thus, valvular heart disease probably exacerbated by hypercoagulability appears to be a source of embolic ischemic brain injury and cerebrovascular disease.
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PMID:Valvular heart disease as a cause of cerebrovascular disease in patients with systemic lupus erythematosus. 1595 May 67

The study was undertaken to determine a possible association of retinal vascular lesion in systemic lupus erythematosus (SLE) with the antiphospholipid syndrome (APS). A hundred and ninety-four patients (160 females and 34 males, whose mean age was 30.7+/-8.9 years) with verified SLE were examined. Group I comprised 67 patients with retinal vascular lesions (Subgroup la (n = 13) with retinal vascular occlusions and Subgroup 1b (n = 54) without occlusions). Group 2 included 127 patients without retinal vascular lesions. APS was detected in 86 patients. Retinal vascular occlusions more frequently occurred in patients with APS (13.9%) than in those without APS (0.9%) (p = 0.0009) and more frequently in patients with APS and thrombocytopenia (24.3%) than in those with APS without thrombocytopenia (6.1%) (p = 0.0359). Extraocular thromboses were more frequently encountered in Subgroup 1a (69.2%) than in Subgroup 1b (33.3%) (p = 0.0399) and in Group 2 (22.8%), (p = 0.0010). Cerebral circulatory disorder (CCD) was observed in 30.7% in Subgroup la, in 14.8% in Subgroup 1b, and in 7.9% in Group 2 (p = 0.0268). Transient ischemic attack (TIA) occurred in 46.2% in Subgroup la, in 24.1% in Subgroup 2a, and in 14.9% in Group 2 (p = 0.0129). Thrombocytopenia was identified in 69.2% in Subgroup 1a, in 22.2% in Subgroup 2a (p = 0.0021), and in 12.6% in Group 2 (p < 0.0000). The frequency of elevated IgG of anticadiolipin (aCL) in Subgroup 1a (80%) exceeded that in Subgroup 1b (37.9%) (p = 0.0309) and Group 2 (30.7%) (p = 0.0096). Another isotype of aCL (IgM 80%) was observed in patients with retinal vascular occlusions, but the differences in this index were insignificant in the groups and subgroups. The association of pathological changes in retinal vessels in the presence of lupus anticoagulant (LA) was particularly noticeable (91.7 and 52% in Subgroup la and Subgroup 1b, respectively (p = 0.0191) and 47.1% in Group 2 (p = 0.0088). There was an association of amaurosis fugax with eyeground occlusions (p < 0.004), CCD and TIA (p < 0.0002), APS (p < 0.0003), and essential hypertension (p < 0.05). Thus, occlusive lesions in the fundus of the eye are a common manifestation of thrombogenesis in SLE in the presence of APS. The frequency of concomitance of retinal vascular occlusions with cerebral circulatory disorders should be referred to as severe manifestations of SLE. Amaurosis fugas is a manifestation of retinal vascular lesion and associated with the presence of APS and elevated blood pressure in patients with SLE. There is an association of an occlusive process in the fundus of the eye with different symptoms of APS and primarily with IgG of aCL, LA, and thrombocytopenia.
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PMID:[Retinal vascular lesions in systemic lupus erythematosus and secondary antiphospholipid syndrome]. 1627 63

Antiphospholipid syndrome is characterized by arterial or venous thrombosis, and the presence of antiphospholipid antibodies (aPL). APL are considered to be a cause of an acquired hypercoagulable state leading to stroke and transient ischemic attack (TIA). We examined the causes in 50 young patients with ischemic stroke. The most prevalent cause was atherosclerosis and the incidence of APS was 12.5%. APL comprise a heterogeneous group of autoantibodies, such as beta2-glycoprotein I dependent anticardiolipin antibody (beta2-GPIaCL), lupus anticoagulant (LA), and other antiphospholid-protein antibodies. We examined the incidence and the pathogenic role of antiphospholipid protein antibodies. The subjects comprised 250 patients (155 male, 95 females) with ischemic stroke, aged 26 to 92 years (mean 72 years). We measured beta2-GPI aCL, IgG aCL, LA, phosphatidyserine dependent antiprothrtombin antibody (PS-PT), antiphosphatidyl-serine antibody (PS), antiphosphatidyl-inositol antibody (PI) in each patient. The incidence of beta2-GPI aCL, IgG aCL, LA, phosphatidyserine, PS-PT, PS, and PI was 2.8%, 12%, 9.2%, 7.2%, 9.6%, and 8.8%, respectively. The incidence of young stroke patients under 50 years was 5.2%. Among 13 young stroke patients, 5 had SLE. Among 23 patients with LA., 18 (78%) patients had PS-PT. Anti-PS-PT antibody is closely related to LA. Antinuclear antibody was detected in 79% of the patients with aPS and/or aPI. We compared the carotid ultrasonographic findings in positive aPI or aPS patients with those in negative ones. Increased IMT, plaque score and carotid stenosis were more common in aPI and aPS-positive patients than in negative ones Three of 5 patients who showed positive beta2-GPI, aCL and LA, simulataneously, had sysyemic lupus erythematosus as an immulological background. Two of 3 patients with PI and/or PS and beta2-GPI and/or LA were patients with SLE. Antiphospholipid antibody was considered to be a risk factor of stroke, especially in SLE and/or young female patients. The incidence of lupus anticoagulant is more common than beta2-GPI aCL in ischemic stroke. In SLE patients with stroke, multi-antiphospholipid-protein antibodies was inclined to be present. LA is closely related to ant-PS-PT and aPI and aPS are associated with anti-nuclear antibody and precipitation of atherosclerosis.
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PMID:[Antiphospholipid syndrome and stroke]. 1644 44

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


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