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)

The antiphospholipid syndrome may present itself to virtually all medical specialties. It can be as non-threatening as thrombophlebitis or mild thrombocytopenia or as severe as cerebral arterial infarct or the so called "catastrophic APS". In order to diagnose it properly, it is important to be aware of its clinical manifestations and laboratory peculiarities. The primary form of APS seems to be more common than the SLE related one. Because any artery or vein may be involved, the spectrum of clinical manifestations of APS is so wide that they include virtually all medical specialties.
Lupus 1996 Oct
PMID:Clinical manifestations of the aPL syndrome. 890 68

Utilizing this unique animal model of thrombosis we demonstrated that human (IgG, IgM or IgA) polyclonal and monoclonal antiphospholipid antibodies derived from APS patients have a significant enhancing effect on thrombus formation. This effect is reversed by treatment of the mice with hydroxychloroquine (plaquenil). In addition murine polyclonal and monoclonal anticardiolipin antibodies induced by active immunization with human beta 2-GP1 or human anticardiolipin antibodies showed to have thrombogenic properties in CD1 mice. Antibodies with antihuman beta 2-GP1 activity alone did not seem to affect thrombus formation.
Lupus 1996 Oct
PMID:In vivo models of thrombosis for the antiphospholipid syndrome. 890 80

During the last few years several murine models for APS have been described. These include spontaneous occurring disease, or APS induced by immunization with pathogenic autoantibodies. Employing those models, several treatment modalities, in different stages of the disease, were studied. Treatments which showed promising potential for application in patients with APS include immunomodulation with specific anti-idiotypic or anti-CD4 antibodies, treatment with IL-3, high-dose intravenous immunoglobulins, ciprofloxacin or bromocriptine, as well as antithrombotic and anticoagulant treatments using aspirin and/or low-molecular-weight heparin. Bone-marrow transplantation was also found to improve clinical and serological manifestations of the disease. These studies might promote the handling of controlled clinical trials assessing their efficacy in APS patients.
Lupus 1996 Oct
PMID:Immunomodulation of experimental APS: lessons from murine models. 890 82

The objective was to study antiphospholipid antibody syndrome (APS or Hughes syndrome) in two major teaching hospitals in Kuwait. patients with suspected Hughes syndrome were investigated with tests for anticardiolipin antibodies (aCL) and lupus anticoagulants (LAC) over 1 yr. Diagnosis was considered confirmed if significant levels of either or both antibodies with no obvious cause (primary), or with systemic lupus erythematosus (SLE) or SLE-like illness (including SLE serology) (secondary) were present. Twelve (37.5%; seven females, 58%) primary and 20 (62.5%; 18 females, 90%) secondary Hughes syndrome patients were seen during this period. patients were Kuwaiti, Middle-Eastern and North-African Arabs (29). Filipinos (2) and White (1). None were from the Indian subcontinent. The main presentation was thrombosis in 75% (arterial in 25% and venous in 50%), and recurrent abortions in 50% of married women. Haematological and dermatological manifestations were limited entirely to the secondary variety, seen in 25% and 19%, respectively. Clinical manifestations were severe, leading to death in one, intensive-care management in 31% and with partial or complete warfarin resistance or brittleness in 25%. Neurological/eye and cardiac manifestations were not seen, as these patients may be attending separate speciality hospitals for these diseases in Kuwait. The approximate prevalence of this syndrome was 2.66/1000 admissions in medical wards. Projected to the total referral areas of the two hospitals, an approximate figure of 52 patients/million population/year was obtained. Hughes syndrome was a common problem among Arabs, Filipinos and possibly Whites in Kuwait. Its manifestations were severe, often requiring intensive-care management, and in one case it was fatal. Patients from the Indian subcontinent were conspicuous by their absence, despite the fact that they were well represented in all other rheumatic disease groups. Ethnic and/or geographical factors could be important in this syndrome. To the best of our knowledge, this is the first report of Hughes syndrome from the Middle East.
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PMID:Hughes syndrome: a common problem in Kuwait hospitals. 894 1

Fetal loss is increased in women who meet the Arthritis and Rheumatism Association criteria for systemic lupus erythematosus (SLE) and in women who have phospholipid antibody syndrome (APS). There are multiple causes for this fetal loss, and in patients with SLE, disease activity appears to be an important contributing factor. In APS patients, it appears that some individuals will experience recurrent fetal loss and will continuously fail to complete pregnancy naturally. Placental examination has helped to elucidate some of the pathology that may be contribute to this fetal loss and our studies have shown that the same pathology is repeated in subsequent pregnancies. Placental examination in SLE or APS patients with recurrent fetal loss is vital if we are going to be able to determine appropriate therapy to prevent fetal loss.
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PMID:Placental pathology in systemic lupus erythematosus and phospholipid antibody syndrome. 903 76

Antiphospholipid antibodies are a wide family of antibodies, dominated by lupus anticoagulant (LA) and anti-cardiolipin antibodies (aCL), encountered in various circumstances. Unnecessary laboratory tests can be avoided by carefully weighing the indications, especially regarding patient age. Three steps are required to demonstrate LA: screening, mixing studies, then confirmation by neutralization tests. Two coagulation tests at least should be performed aCL are detected with ELISA kits using plates coated with cardiolipin. Due to the large number of kits available and to the lack of agreement on cut-off values, all laboratories must indicate their own standards. Other kits use plates coated with a mixture of phospholipids. Recent data suggest that pathogenic aPL are more specifically directed against phospholipid-associated proteins rather than towards phospholipids. In the future, tests for aCL might be replaced by tests for beta 2-glycoprotein I. The presence of aPL requires a specific treatment only in patients presenting clinical manifestations thought to be aPL-induced (thromboses, fetal losses). Long term warfarin aimed at an INR of 3-3.5 is effective for the secondary prevention of thrombosis. In primary APS, prevention of recurrent miscarriages is frequently achieved by a combination of subcutaneous heparin plus aspirin.
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PMID:[Antiphospholipid antibody/cofactors. What are they? Why, when and how to search for them? Is treatment justified?]. 909 60

aPL antibodies are a wide and heterogeneous family of autoantibodies, formerly believed to be directed at anionic phospholipids. In recent years they have been shown to be directed at plasma proteins bound to suitable (phospholipid) anionic surface: beta 2-GPI and prothrombin are the best known and characterized antigens, which are recognized by aCL antibodies and most Lupus Anticoagulants, respectively. The presence of these antibodies has been associated with arterial and venous thrombosis, recurrent miscarriages and thrombocytopenia in the so-called "Antiphospholipid Syndrome". Retrospective and "cross-sectional" studies have established the role of aCL antibodies and Lupus Anticoagulants as risk factors for both venous and arterial thrombosis, the most common clinical manifestations of APS. Prospective studies performed in different patients' populations have validated the association between aCL antibodies and Lupus Anticoagulants with venous and, possibly, arterial thrombosis. Along with the concept of the heterogenity of aPL antibodies there is the observation that among Lupus Anticoagulants aCL-type A, but not LA antibodies, appear to represent a risk factor for thrombosis. However, informations on the predictive value of the various laboratory tests with respect to thrombosis are still rather limited. It is, therefore, necessary to continue the development and standardization of assays that selectively identify aPL antibodies associated with an increased risk of thrombosis, in order to help the clinicians to establish the most appropriate therapeutic strategies for the prevention of the thromboembolic complication of APS.
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PMID:Antiphospholipid antibodies: predictive value of laboratory tests. 919 31

The etiology of autoimmune diseases is multifactorial. In many of them the stimulation by a specific autoantigen is claimed to be responsible for the initiation of the disease. Alternatively, an autoimmune state may be induced by a pure dysregulation of the immune system. Such is the case in which severe systemic lupus erythematosus (SLE) is induced in young patients with myasthenia gravis following thymectomy. We have referred to this set of events as the 'kaleidoscope of autoimmunity'. Herewith, we would like to present another example of the kaleidoscope phenomenon, namely: the emergence of a full blown clinical presentation of the primary antiphospholipid syndrome (APS-recurrent thromboembolic phenomena, repeated fetal loss with high titers of anti-cardiolipid antibodies) in a 32 y old female with myasthenia gravis, two years following thymectomy. Thymectomy in myasthenic patients may be associated with the emergence of new autoimmune conditions such as SLE and APS, pointing to the importance of immune dysregulation in the induction of these autoimmune diseases.
Lupus 1997
PMID:Primary antiphospholipid syndrome emerging following thymectomy for myasthenia gravis: additional evidence for the kaleidoscope of autoimmunity. 928 13

The aim of this study was to characterize the antigen specificity and to evaluate the diagnostic and prognostic value of anti-mitochondrial M5 type antibodies (AMA M5). Fifty-eight patients selected on the basis of their AMA M5 positivity were investigated in relationship to their clinical and serological profile. Cross-absorption studies, Western blotting and immunoprecipitation analysis were carried out for AMA M5 antigen specificity characterization. Most patients had a diagnosis of systemic lupus erythematosus (SLE) (65.5%) or of primary anti-phospholipid syndrome (PAPS) (24%); all the patients were positive for IgG or IgM anti-cardiolipin (anti-CL) antibodies and 49% of them also displayed lupus anticoagulant (LA) activity. Anti-beta2-glycoprotein I (beta2-GPI) IgG were detectable in 30/38 sera (78.9%) and IgM in 34/38 (89.4%). While anti-CL and anti-beta2-GPI IgG antibodies were significantly associated with history of thrombosis and fetal loss, AMA M5 displayed a statistical association only for thrombocytopenia and recurrent fetal loss. Absorption with human beta2-GPI both in free solution or in solid phase as well as with CL liposomes or CL/beta2-GPI liposome complexes did not affect AMA M5 fluorescence. While AMA M5 activity is absorbed by whole mitochondrial preparations, no specific reactivities against several human, bovine and rat mitochondrial proteins could be detected in Western blotting and immunoprecipitation studies. AMA M5 appear to be detectable in both primary and secondary APS, displaying a strong association with the presence of thrombocytopenia and fetal loss. Although strictly related to anti-phospholipid antibodies, AMA M5, anti-CL and anti-beta2-GPI antibodies represent distinct serological markers of the APS.
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PMID:Anti-mitochondrial M5 type antibody represents one of the serological markers for anti-phospholipid syndrome distinct from anti-cardiolipin and anti-beta2-glycoprotein I antibodies. 956 3

A role for Fcgamma receptor in the pathophysiology of thrombosis in APS has been hypothesized. The polymorphism of this receptor, FcgammaRIIA H/R131, is associated with the binding affinity for human IgG2 (i.e. FcgammaRIIA-H131 isoform has a higher affinity than FcgammaRIIA-R131). Since anti-beta2 glycoprotein I antibodies (anti beta2GPI), which play a major pathogenic role in APS, show IgG2 dominant distribution, we investigated the prevalence of receptor isoforms in patients with antiphospholipid antibodies (aPL) by a PCR-RFLP method. We studied 100 Caucasian patients with aPL (57 primary APS, 32 secondary APS to SLE and 11 other diseases with aPL) and 41 healthy controls. H131/H131, H131/R131 and R131/R131 genotypes were found in 21 (21%), 50 (50%) and 29 (29%) in the patient group, and 9 (22%), 23 (56%) and 9 (22%) in control group, respectively. Thus there was no statistically significant difference in the prevalence of each genotype in these groups. None of the clinical manifestations of primary APS (arterial/venous thrombosis, recurrent pregnancy loss and thrombocytopenia) was significantly correlated with any FcgammaRIIA genotype. In conclusion, FcgammaRIIA polymorphism did not correlate with the manifestations of APS, and FcgammaRIIA genotype is not a genetic marker of APS.
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PMID:Fcgamma receptor IIA H/R131 polymorphism in patients with antiphospholipid antibodies. 960 22


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