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 main objectives of the present study were to determine the prevalence of IgG and IgM anticardiolipin antibody (aCL) isotypes in systemic lupus erythematosus (SLE) in order to analyze their possible association with the clinical manifestations of the disease. Clinical features of 64 consecutive and unselected SLE patients were prospectively studied. Sera from the same patients taken during each clinical manifestation were tested for the presence of aCL. The prevalence of aCL was 43.75% for the IgG isotype and 9.4% for the IgM isotype. A strong linkage between the presence of these antibodies and the occurrence of both thrombosis and abortions was found: a weaker association with neurological events and thrombocytopenia was also demonstrated. The titre of aCL appeared to be linked with the probability of having the clinical manifestations associated with these autoantibodies. Our results suggest that thrombosis and abortion, and possibly thrombocytopenia and central nervous system involvement, may be associated with the presence of aCL at the time when these clinical events develop in SLE patients.
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PMID:Prevalence and clinical associations of anticardiolipin antibodies in systemic lupus erythematosus: a prospective study. 179 Jun 38

The antiphospholipid antibody syndrome (APLAS) is a unique clinical syndrome with features of recurrent thrombosis, recurrent fetal loss, and thrombocytopenia. It is associated with a false positive test for syphilis, a prolonged partial thromboplastin time (PTT), a positive test for lupus anticoagulant (LA), and anticardiolipin antibodies (ACLA). A case report illustrating some of the clinical and laboratory abnormalities and therapeutic dilemmas is presented. The literature is then reviewed.
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PMID:Antiphospholipid antibody syndrome: a case report and review of the literature. 180 Nov 88

We report a case of a 47-year-old man with anti-phospholipid antibody syndrome associated with subdural hematoma. The patient had several episodes of arthritis during his thirties. He developed venous thrombosis in his right lower leg at the age of 35, when laboratory studies demonstrated prolongation of activated partial thromboplastin time (APTT) and a biological false-positive result occurred on a syphilis test. When bulbar palsy and Horner's syndrome in the right eye suddenly appeared at the age of 42, he was diagnosed as having brain stem infarction. At the age of 47, he developed constructional apraxia, dyscalculia, skilled movement disturbances and generalized convulsions. Subdural hematoma and multiple lacunes in the cerebral white matter were demonstrated with brain MRI. Furthermore, the patient was positive for Rumpel-Leede phenomenon. Laboratory studies revealed mild thrombocytopenia, prolonged bleeding time and APTT, positive antinuclear antibody and positive test results for both lupus anticoagulant and an anti-cardiolipin antibody, namely anti-phospholipid antibodies. Based on these findings, we consider that the tendency of this patient to bleed may have been due to antiphospholipid antibodies, attacking the platelet membranes and that the bridging veins in the subdural space may be the site at which the bleeding tendency easily appears. Anti-phospholipid antibody syndrome accompanied by hemorrhagic complications had rarely been reported. We suggest that special attention should be given to hemorrhagic complications in patients with anti-phospholipid antibody syndrome associated with fragility of the vessels and/or platelet dysfunction.
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PMID:[A case of anti-phospholipid antibody syndrome associated with subdural hematoma]. 180 71

Seven patients with systemic lupus erythematosus (SLE), persistent thrombocytopenia (TP), in whom it was considered undesirable to institute an increase in steroid or immunosuppressive agents, were treated with danazol. Five patients completed the minimum period of 8 weeks. Two patients showed early response to danazol but were switched over to cyclophosphamide or azathioprine after 4 weeks because of systemic disease. Of the remaining five patients, four had complete responses. In one patient who failed treatment the TP was considered to be related to another drug (ranitidine). Other manifestations of SLE also improved with treatment. Side effects included amenorrhea in one patient, and hypoglycemia and hyponatremia in another. Infections were absent. Danazol can be a useful alternative treatment of lupus TP.
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PMID:Danazol in treatment of lupus thrombocytopenia. 180 60

Anti-cardiolipin antibodies have been recently described in patients with systemic lupus erythematosus and other autoimmune disorders. Venous and arterial thrombosis, thrombocytopenia and recurrent abortion have been associated to the presence of these antibodies. Among 161 patients with systemic lupus we found 66% with anticardiolipin antibodies, mostly IgG type. Thrombosis and thrombocytopenia were more frequent in these patients (p < 0.05), especially among those with high titers. No association was found with central nervous system involvement, recurrent abortion, antinuclear or anti-DNA antibodies nor positivity for VDRL. These results support the frequent presence of anti-cardiolipin antibodies in patients with systemic lupus and their association to thrombosis and thrombocytopenia.
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PMID:[Anticardiolipin antibodies in systemic lupus erythematosus: prevalence and clinical associations]. 184 89

Recently, the association between anti-phospholipid antibodies (false positive VDRL, lupus anticoagulant or anti-cardiolipin antibody) and diverse clinical manifestations has been termed antiphospholipid syndrome. We report 6 female patients with "primary" antiphospholipid syndrome, not related to connective tissue disorders. Their age ranged from 23 to 66 years and they were followed from 1 to 27 years (mean 9.2). Venous occlusion developed in 4, arterial occlusion in 4 (TIA, convulsive episode and cutaneous thrombotic microangiopathy). Three of 5 had fetal loss and 3/6 developed thrombocytopenia. Leg ulcer, migraine and mitral valvulopathy and peripheral facial paralysis were isolated manifestations in different patients. High titers for type IgG anticardiolipin antibodies were present in all patients. Low titers for IgM antibodies were present in 2. The pathogenesis of this syndrome is discussed.
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PMID:[Primary antiphospholipid syndrome: clinical experience of 6 patients]. 184 92

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.
Lupus 1991 Nov
PMID:Neonatal lupus erythematosus with multisystem organ involvement preceding cutaneous lesions. 184 64

Having reviewed the literature on the association of aPL antibodies with clinical manifestations, it is clear that this group of autoantibodies are of considerable importance. The presence of aPL antibodies in some but not all individuals confers a risk of a clinical syndrome characterized by recurrent arterial or venous thrombosis, thrombocytopenia, hemolytic anemia, or positive Coombs' test, and in females, recurrent idiopathic fetal loss. In SLE, the risk is approximately 40%, compared with a risk of 15% in the absence of aPL antibodies. However, only one half of persons possessing these antibodies have SLE, and overall the risk is around 30%. In some circumstances, such as in chlorpromazine or infection-associated aPL antibodies, there appears to be no increased risk. At the other end of the spectrum are seen patients whose only clinical manifestations comprise features of this clinical syndrome, and this entity has been designated the primary antiphospholipid syndrome (PAPS). aPL antibodies are also important because they are not uncommon. They have been found frequently in women with idiopathic recurrent fetal loss (30%), in non-autoimmune patients with ischemic heart disease (20%), or venous thrombosis (up to 30%), or stroke (4-47%), and in chronic immune thrombocytopenia (30%). These autoantibodies can be detected using sensitive solid-phase immunoassays employing the CL antigen, or in appropriate coagulation tests to detect LA activity. These assays are simple to perform but require care in selection of the best test and in interpretation of results. Current tests do not distinguish between those persons at risk of the clinical events and those not at risk. Detection of specific isotypes (especially IgG) and antibody level may aid in such a designation. Treatment of aPL antibody-associated syndromes remains a controversial subject. Since thromboses are associated with significant morbidity and potential mortality, there is a good argument for long-term preventive antithrombotic therapy, at least for as long as the antibodies are detectable, in those patients in whom clinical complications have previously occurred. It is not generally recommended that this treatment be offered to individuals in whom aPL antibodies are detected but who have not suffered previous thromboses, since the risk of such events does not appear to be equal within a group of aPL antibody-positive persons. This particularly applies to pregnant women, since live births and uncomplicated pregnancies are observed regularly in the presence of aPL antibodies without specific treatment. A previous history of at least one unexplained, late fetal loss is considered a prerequisite before intervention in subsequent pregnancies.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Immunology and clinical importance of antiphospholipid antibodies. 185 85

The human monoclonal autoantibody HF2-1/17, produced by a human-human hybridoma derived from lymphocytes of a lupus patient with thrombocytopenia, reacts with single stranded DNA and platelets. To determine the chemical nature of the autoantigen against which this antibody is directed on platelets, this platelet antigen was purified by the lipid extraction of sonicated platelets, DEAE-Sephadex chromatography, and high performance liquid chromatography. The purified glycolipids, a trace component in platelets, demonstrated high reactivity with the HF2-1/17 antibody using a competition enzyme-linked immunosorbent assay system or immunostaining of thin layer chromatograms. The purified glycolipids co-migrated with bovine sulfatides by thin layer chromatography. The purified glycolipids contain sulfate and galactose but not sialic acid or phosphate. Fast atom bombardment-mass spectrometry revealed these sulfatides to be sulfated monohexyl ceramides. The dominant species has a molecular weight of 794 while a minor form has a molecular weight of 812 due to an extra hydroxyl group and loss of a double bond. These results indicate that the platelet autoantigen against which the human monoclonal anti-DNA antibody is directed represents a family of novel monogalactosyl sulfatides.
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PMID:Sulfated glycolipids are the platelet autoantigens for human platelet-binding monoclonal anti-DNA autoantibodies. 186 60

Antibodies directed to a co-factor associated with negatively charged phospholipids, such as cardiolipin, occur in patients with systemic lupus erythematosus (SLE), and possibly more often in those with venous or arterial thrombosis, thrombocytopenia or recurrent fetal loss. They are also found in patients without any of these manifestations and their biological effect, if any, might thus be related to their IgG subclass. To investigate this possibility, we determined anticardiolipin antibodies (ACA) by enzyme immunoassay (EIA) using monoclonal antibodies (MoAb) against human IgG subclasses. A net absorbance of x +3 SD of the value of 30 blood donors was taken as the cut-off point. The specificity of the assay was verified through inhibition experiments using cardiolipin micelles. Thirty-three patients with SLE were studied, all of whom had been shown to have ACA by a point dilution screening assay. IgG1 ACA were found in 85% of the patients, and ACA of the IgG2, IgG3 and IgG4 subclasses in 42%, 39% and 15%. There was a significant correlation between the presence of IgG3 ACA and of anti-DNA antibodies but none between subclass distribution and major clinical manifestations of SLE.
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PMID:Subclasses of IgG anticardiolipin antibodies in patients with systemic lupus erythematosus. 188 85


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