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)

Many consensus meetings were organized in an attempt to improve the present criteria for antiphospholipid syndrome (APS) classification. In this regard, a high prevalence of antiphospholipid antibodies in systemic lupus erythematosus patients was reported in association with the presence of livedo reticularis (LR). In these studies, the association between LR, migraine, and the development of thrombosis (strokes, valvular dysfunctions) was evident. During the last decade, it was strongly suggested that many clinical symptoms (LR, valvular dysfunctions) or laboratory features (thrombocytopenia) should be considered as "minor criteria" for APS. The inclusion of these clinical symptoms in the criteria for APS classification could become of additive value especially when they exist together in one patient. This review summarizes the data that question or support this idea.
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PMID:Livedo reticularis as a criterion for antiphospholipid syndrome. 1791 83

Stroke constitutes a serious socioeconomic and health care problem in women because it is the principal cause of incapacity and the first cause of death in this section of the population in several countries. The incidence of cerebral infarction and myocardial infarction is lower in females than in males, but only up to menopause, when, especially after >65 years of age, the differences disappear. This is attributable to the protective role of the natural estrogens during the fertile part of the woman's life. The estrogens have antiatherogenic and neuroprotective effects and, before menopause, account for a risk profile that is less atherogenic. The risk factors that have higher importance in young women are migraine and oral contraceptive use as well as etiologies specifically associated with pregnancy, birth and puerperium, or even diseases that present more commonly in women (systemic lupus erythematosus, fibromuscular dysplasia). These entities lose their relevance after menopause, when the classical vascular-disease risk factors take on their role, and also include others such as hormone replacement therapy. Due to the specific epidemiology of stroke, females are underrepresented in the clinical trials of primary and secondary prevention, the result being that the outcomes measured in these trials are less consistent and do not show as clear a benefit for women as for men.
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PMID:Peculiarities of stroke risk in women. 1797 41

The involvement of the central nervous system (CNS) is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients and the less understood aspect of the disease. Its recognition and treatment continue to represent a major diagnostic and therapeutic challenge. Due to the lack of controlled randomized trials, current therapeutic approach is still empirical and based on clinical experience. The therapeutic choice depends on accurate diagnosis, identification of underlying pathogenic mechanism, severity of the presenting neuropsychiatric symptoms, and on prompt identification and management of contributing causes of CNS disease. Mild neuropsychiatric manifestations may need symptomatic treatment only. In more severe CNS disease it is important to distinguish between thrombotic and non-thrombotic mechanisms. Focal CNS manifestations, particularly TIA and stroke, are associated with the presence of antiphospholipid antibodies (aPL). Anticoagulation is warranted in patients with thrombotic disease, particularly in those with the antiphospholipid (Hughes) syndrome (APS). Other CNS manifestations, such as demyelinating syndrome, transverse myelitis, chorea, seizures, migraine and/or cognitive dysfunction, when associated with persistent positivity for aPL, may also benefit from anticoagulation in selected patients. Severe diffuse CNS manifestations, such as acute confusional state, generalised seizures, mood disorders and psychosis, generally require corticosteroids in the first instance. Pulse intravenous cyclophosphamide therapy may help when more severe manifestations are refractory to corticosteroids and other immunosuppressive agents, generally when response is not seen in 3-5 days. Plasmapheresis may also be added in severe cases of symptoms refractory to conventional treatment. Intravenous immunoglobulins, mycophenolate mofetil, rituximab, intratecal methotrexate and dexametasone deserve further studies to confirm their usefulness in the treatment of neuropsychiatric SLE. This article reviews the clinical approach to therapy in patients with SLE and neuropsychiatric involvement.
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PMID:Neuropsychiatric involvement in systemic lupus erythematosus: current therapeutic approach. 1853 50

The aim of our study was to evaluate the clinical and HLA-class II allele associations of some anti-cofactor antibodies in a homogeneous group of European patients with SLE. One hundred thirty-six patients with SLE, fulfilling four or more of the ACR 1997 revised criteria for the classification of the disease, coming from 7 European countries, were enrolled consecutively. Anti-prothrombin (anti-PT), anti-annexin V (anti-AnnV), anti-protein C (anti-Cprot) and anti-protein S (anti-Sprot) were determined by using commercial ELISA kits. Molecular typing of HLA-DRB1, DRB3, DRB4, DRB5, DQA1, DQB1 and DPB1 loci was performed by using PCR-SSOP method, carried out using digoxygenin (DIG) labeled probes. The prevalence of anti-AnnV, anti-PT, anti-Cprot and anti-Sprot was 19%, 10.4%, 4.4% and 8.1%, respectively. Twenty-seven % of anti-AnnV positive patients reported migraine vs 5.5% of anti-AnnV negatives (p = 0.003, but p not significant, odds ratio (OR) = 6.4, 95% confidence interval (CI) = 2-21). Anti-PT, anti-AnnV and anti-Sprot were positively associated with some HLA alleles, but pc was not significant. In this study we have shown that some HLA alleles carry the risk to produce antibodies against phospholipid-binding proteins, but these association need confirmation in other studies, because they have never been reported and appear to be weak associations.
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PMID:Anti-cofactor autoantibodies in systemic lupus erythematosus: prevalence, clinical and HLA class II associations. 1856 76

Young patients with migraine are at increased risk for stroke, particularly patients with an aura of focal neurologic deficits. Other causes of ischemia are often identified in patients with migraine, including patent foramen ovale, lupus anticoagulant, cervical carotid dissection, arteriovenous malformation, and hyperactivity of the clotting system. Migrainous stroke is only diagnosed when all other possible causes of stroke have been eliminated and the patient has irreversibility of the usual aura, associated with an ischemic infarct in the appropriate brain territory. Prophylactic therapy of migraine with aura may be beneficial in preventing migrainous stroke.
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PMID:Stroke and migraine. 1858 14

The antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPL), associated with thrombosis or recurrent spontaneous abortions. APS can occur alone or secondary to other conditions, especially associated to inflammatory systemic autoimmune diseases. Among the neurological manifestations associated with aPL, only ischemic stroke is recognized by the actual classification criteria for APS. Other neurological manifestations have been, however, repeatedly reported in case studies of APS patients. Headache, and especially migraine, was commonly reported in APS patients and is one of the classical features described by Hughes as related to aPL, but studies failed to confirm this association. We studied retrospectively the association between headache syndromes and aPL in 428 patients with inflammatory connective tissue diseases admitted in the Neurology and Internal Medicine Departments of Colentina Hospital-Bucharest. We found that migraine alone, not headache of all types, is significantly associated with aPL in patients with systemic immune disease. We studied the presence of cerebral ischemia in patients with headache and aPL. In SLE patients, headache (all types) is significantly associated with positive titers of aPL, and cerebral ischemic lesions are significantly encountered. Even if both migraine and aPL are conditions with high frequency in patients with immune systemic disease and their association may be coincidental, the presence of ischemic lesions in patients showing this association suggests the need to define a sub-group at risk, for whom headache can be a marker and anticoagulants can be discussed.
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PMID:Antiphospholipid antibodies and migraine: a retrospective study of 428 patients with inflammatory connective tissue diseases. 1876 11

Controversy exists as to whether patients with migraine may suffer cortical damage. We investigate the possible association between impaired cognitive function and chronic headache in lupus patients. Sixty one patients with systemic lupus erythematosus but without the antiphospholipid (Hughes) syndrome were questioned about headaches and formally assessed for cognitive function. They were also subjected to magnetic resonance imaging (MRI). Twenty one patients denied any significant headaches, 19 reported migrainous headaches and 11 experienced an aura. Eleven patients experienced headaches with features of migraine but did not fulfill the criteria, and seven patients had tension headaches. All patients had stable lupus; there was no difference in the incidence of hypertension, age or previous episodes of neuropsychiatric lupus. Patients with migrainous headaches without aura had marginally shorter duration of disease. There was no difference between the groups with respect to eight different cognitive tests or the ventriculo brain index on MRI. We failed to detect cognitive impairment in lupus patients with chronic headaches including migrainous headaches.
Lupus 2009 Jun
PMID:An investigation in the possible effect of chronic headache on neuropsychological function in aCL-negative patients with SLE. 1943 61

The objectives of the study were to describe the experience of patients immediately prior to a diagnosis of Hughes syndrome (HS) or antiphospholipid syndrome and post-diagnosis. A questionnaire survey was carried out set in the Hughes Syndrome Foundation, St. Thomas' Hospital, London, 2006. Participants were all patients who are members of the Hughes Syndrome Foundation. The main outcome measures were responses to a questionnaire relating to the experiences of people with a diagnosis of HS, such as number of hospitalisations, number of consultants seen, number of miscarriages, etc. A total of 157 patients completed the questionnaire, giving a response rate of 60.4%. Most (85%) were women and mean age was 46 years (SD 12). The median time to diagnosis was 3 years. The median number of consultants seen was 2 (max 19) with a median time in hospital pre-diagnosis of 10 days. The most common initial diagnoses were migraines, multiple sclerosis and systemic lupus erythematosus. Among women, 46% had had a miscarriage. Two thirds of respondents thought a blood test would have led to an earlier diagnosis. Comments from patients indicated a lack of awareness among specialists and general practitioners. The survey demonstrated a long time lag for diagnosis of Hughes syndrome, with increased costs to the NHS and emotional and financial cost to the patient. Greater awareness of this condition would benefit patients and the NHS.
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PMID:Patients' experiences of a diagnosis of Hughes' syndrome. 1945 65

The Euro-Phospholipid project started in 1999 with a multicentre, consecutive and prospective design. A total cohort of 1000 patients with antiphospholipid syndrome (APS), derived from 13 countries (Belgium, Bulgaria, Denmark, France, Germany, Greece, Hungary, Israel, Italy, the Netherlands, Portugal, Spain and United Kingdom), has been followed since then. This project allowed the identification of the prevalence and characteristics of the main clinical and immunological manifestations at the onset and during the evolution of APS and demonstrated that it is possible to recognize more homogeneous subsets of clinical significance. Patients with APS associated with systemic lupus erythematosus (SLE) had more episodes of arthritis, livedo reticularis and more frequently exhibited thrombocytopenia and leucopenia. Female patients had more episodes of arthritis and livedo reticularis - both connected with the higher prevalence of migraine and SLE-related APS in women, while male patients had more myocardial infarction, epilepsy and lower limb arterial thrombosis. Childhood onset patients presented more episodes of chorea and jugular vein thrombosis, whereas older onset patients were more frequently male and had more strokes and angina pectoris, but less frequently livedo reticularis.
Lupus 2009 Sep
PMID:The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe. 1967 88

Current diagnostic classification criteria recommend elevated titres of anti-cardiolipin (aCL) and/or anti-beta(2)GPI antibody by ELISA IgG or IgM and/or lupus anticoagulant (LA) to confirm antiphospholipid syndrome (APS). Although IgA aPL antibodies have been shown to be pathogenic in animal models of APS, their clinical significance has remained elusive. We report four cases of exclusive IgA anti-beta(2)GPI antibody sero-positivity with concomitant clinical manifestations associated with APS. Four of the five patients were LA negative. 1) Thirty-eight-year-old African-American female with SLE presented with resolving digital ulcers. Serum IgA anti-beta(2)GPI antibody titres were 118.5 SAU (normal range: 0-20 SAU). 2) Twenty-seven-year-old African-American woman with SLE was evaluated for recent onset of severe headaches, unresponsive to analgesics and anti-migraine medications. MRI of the brain revealed hyper-intensities in the white matter in the frontal lobes. Serum IgA anti-beta(2)GPI antibody titres were 29.1 Standard A Units (SAU). 3) Thirty-two-year-old Hispanic female with history of two unexplained miscarriages and negative serologies for SLE. Serum IgA anti-beta(2)GPI antibody titres were 102.0 SAU. 4) Twenty-five-year-old white female with history of recent unexplained miscarriage in the 11th week of gestation and associated complaints of numbness and tingling in her hands. Her IgA anti-beta(2)GPI antibody titre was 62.0 SAU. 5) Twenty-five-year-old African-American woman with SLE, positive for anti-Ro antibodies with a history of ischemic fingers, a pregnancy loss and recent pregnancy complicated due to pre-eclampsia. Her LA was positive and her IgA anti-beta(2)GPI antibody titer was 186.0 SAU. This case series supports that elevated IgA anti-beta(2)GPI antibody titres may identify additional patients who have clinical features of APS but who do not meet current diagnostic criteria.
Lupus 2009 Oct
PMID:Isolated elevation of IgA anti-beta2glycoprotein I antibodies with manifestations of antiphospholipid syndrome: a case series of five patients. 1976 4


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