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 association of anticardiolipin antibodies (aCL) with unexplained vascular occlusive disease (VOD) is well known. We reviewed the records of 102 consecutive patients seen over a 9 months period who had positive IgG or IgM aCL to determine the frequency and types of VOD in this unselected group of patients. Lupus anticoagulant was detectable in 17 of 67 (25%) patients tested. VOD occurred in 80 of 102 (78%) aCL-positive patients comprised of 17 (16.7%) with systemic venous VOD or pulmonary embolism; 27 (26.5%) with cerebral VOD: 11 (10.8%) with systemic arterial VOD; 3 (2.9%) with coronary thrombosis; and 5 (4.9%) with visceral venous or arterial VOD. Of the 19 obstetric patients with positive aCL, 17 (89%) had at least one unexplained fetal loss and 8 of the 17 (47%) had multiple or recurrent fetal losses. Twelve (11.7%) of the 102 patients met the ACR criteria for systemic lupus erythematosus (SLE). Additionally, 12 (11.7%) patients were identified as nonSLE or undifferentiated connective tissue disease (CTD). The remaining 78 (76%) had no known underlying disease (primary antiphospholipid syndrome). We conclude that IgG and IgM aCL with or without lupus anticoagulant are associated with diverse types of VOD but cerebral VOD appears predominant. aCL-associated unexplained VOD occurs frequently in patients without evidence of CTD-65 of 80 (81%) in our series. Testing for aCL is essential for identifying patients with unexplained VOD, and it should be performed in prospective clinical studies of such patients to better define the pathogenic role of aCL in the natural history of unexplained VOD.
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PMID:The prevalence of vascular occlusive disease associated with antiphospholipid syndromes. 152 53

An 8-year-old black girl with a 5 month history of systemic lupus erythematosus (SLE) and secondary antiphospholipid syndrome (APS) developed Raynaud's phenomenon, marked hemolytic anemia, and a fatal myocardial infarction (MI). Pathologic evaluation of the heart demonstrated a transmural acute MI associated with a recent thrombus of the circumflex coronary artery, thrombosis of small intramural arteries, and a coronary arteriopathy resembling fibromuscular dysplasia. Inflammatory or atherosclerotic changes of the coronary arteries were distinctly absent. This case represents the youngest reported patient with SLE, MI, and pathologic confirmation of nonatheromatous coronary artery disease. The observed coronary pathological findings may have accentuated the thrombogenic potential of the APS, resulting in coronary thrombosis. Cardiac lesions in SLE and APS are reviewed, and pathogenetic considerations for the coronary vasculopathy are discussed.
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PMID:Fatal myocardial infarction in an 8-year-old girl with systemic lupus erythematosus, Raynaud's phenomenon, and secondary antiphospholipid antibody syndrome. 779 Nov 80

Antiphospholipid antibodies are strongly associated with thrombosis and appear to be the most common of the acquired blood protein defects causing thrombosis. Based upon our experience, approximately 25% of patients with unexplained venous thrombosis, approximately 60% of patients with cerebrovascular thrombosis, approximately 37% of patients with transient ischemic attacks, approximately 18% with premature coronary artery thrombosis and approximately 60% of patients with recurrent fetal loss (recurrent miscarriage syndrome) harbor antiphospholipid antibodies. Although the precise mechanism(s) whereby antiphospholipid antibodies alter hemostasis to induce a hypercoagulable state remain unclear, several theories have been advanced. Since the aPTT is unreliable in patients with lupus anticoagulant and is not usually prolonged in patients with anticardiolipin antibodies, definitive tests, ELISA for IgG, IgA and IgM anticardiolipin antibodies and the dilute Russel's viper venom time (followed by cephalin correction for confirmation) for lupus anticoagulant should be immediately ordered when suspecting the antiphospholipid syndrome in individuals with otherwise unexplained thrombotic or thromboembolic events or recurrent fetal loss. However, if one strongly suspects antiphospholipid thrombosis syndrome clinically and assays for lupus anticoagulants and anticardiolipin antibodies are negative, specific assays for all three idiotypes of phosphatidylserine, phosphatidylethanolamine, phosphatidylcholine, phosphatidylinositol and phosphatidylglycerol are available and should be considered. These may clearly be indicated for difficult diagnostic cases of fetal wastage syndrome, and cerebrovascular events, but their significance in other types of thrombosis, particularly venous, remains unclear at present. Since about 65% of patients with antiphospholipid antibodies will fail warfarin therapy (rethrombose), it is important to define this common defect and institute appropriate antithrombotic therapy for appropriate time periods.
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PMID:Antiphospholipid-thrombosis syndromes. 1062 91

The antiphospholipid antibody syndrome (APLAS), though an uncommon entity involves multiple organs in the body. The antiphospholipid antibodies (APLA) refer to several groups of autoantibodies against negatively charged phospholipids occurring independently or in association with systemic lupus erythematosus (SLE) and related autoimmune disorders. Several studies to date found those patients with APLA, predominantly IgG and to lesser extent IgM isotype and lupus anticoagulant (LAC) are associated with arterial and venous thrombosis, recurrent fetal loss, thrombocytopenia, and livedo reticularis. We have described two cases of APLAS, one primary and the other secondary, their management and cardiac manifestations. Cardiac manifestations of the syndrome include coronary artery thrombosis and valvular heart disease. These can be serious and difficult to treat. Although the exact treatment of the cardiac manifestations of APLAS is not clear, anticoagulation is the currently recommended therapy.
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PMID:Cardiac manifestations of the antiphospholipid antibody syndrome: a review. 1197 83

NZW "x" BXSB F1 mice develop SLE that is associated with an anti-phospholipid syndrome characterized by anti-cardiolipin antibodies, thrombocytopenia and small coronary artery thrombosis. This syndrome is immune mediated and, dependent, on CD4+T cells. To determine whether disease in these mice can be treated with blockade of T cell costimulation we treated them with the CD28 antagonist CTLA4Ig at 9 or 12 weeks of age. CTLA4Ig completely prevented both SLE nephritis and myocardial infarcts if it was given at 9 weeks of age, before anti-cardiolipin antibodies could be detected in the serum and prevented both B cell expansion and activation and the development of peripheral monocytosis. If treatment was delayed until 12 weeks of age after cardiolipin antibodies had arisen but before the onset of clinical disease, CTLA4Ig had very little effect on disease progression. These findings indicate that CD4+T cell activation through CD28 is critical for disease initiation in this model but plays little role in disease progression or tissue damage. These findings have relevance to the treatment of anti-phospholipid syndrome in humans.
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PMID:CTLA4Ig prevents initiation but not evolution of anti-phospholipid syndrome in NZW/BXSB mice. 1562 70

In our article we describe the case of 24 years old woman with overlap syndrome under form of systemic sclerosis and systemic lupus erythematosus complicated by secondary antiphospholipid syndrome (APS). The first manifestation of antiphospholipid syndrome was intrauterine fetal death. Afterwards pulmonary embolism occurred. After several weeks in result of catastrophic course of antiphospholipid syndrome coronary artery thrombosis and myocardial infarction occurred with following prominent left ventricle systolic failure and multi organ failure. The patient died about one month after discharge from the hospital.
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PMID:[Secondary lethal catastrophic antiphospholipid syndrome in 24-years old female patient with overlap syndrome (systemic sclerosis and systemic lupus erythematosus)]. 1678 Feb 70

The present study aims to report a-20-year old girl with systemic lupus erythematosus (SLE) who developed myocardial infarction (MI) and also aims to review acute myocardial infarction (AMI) in young SLE cases (< or =35 years) reported in the literature. We conducted a comprehensive review of the English literature from 1975 to 2006 to analyse data on MI in SLE patients who had developed AMI either at 35 or earlier. In 32 English articles, we identified 49 SLE patients, plus our case, with AMI. They consist of 41 female and nine male patients, their mean age being 24 +/- 6.4 years (range of 5-35). Disease duration varied between 0 and 13 years. The lag time between the onset of the SLE manifestations and development of AMI was 7.7 +/- 5.4 year (range of 1 month to 20.5 years). We divided the patients into three subgroups according to their coronary involvement type (Group I: normal coronary artery or coronary thrombosis (n = 16); Group II: coronary aneurysm/arteritis (n = 12); Group III: coronary atherosclerosis (n = 22)). The lag time between the onset of the SLE manifestations and development of MI in the subgroups showed variations: Group I < Group II < Group III. Both prevalence of renal involvement and steroid therapy were higher in patients with coronary atherosclerosis than were in Group I. There were one or more risk factors for atherosclerosis in 39 SLE patients. AMI in young SLE patients may be seen, albeit rare. We suggest that clinicians should have a low threshold for cardiac evaluation in patients with SLE. Also, traditional risk factors could be managed through preventive measures.
Lupus 2007
PMID:Myocardial infarction in young patients (< or =35 years of age) with systemic lupus erythematosus: a case report and clinical analysis of the literature. 1743 37

Myocardial infarction (MI) is a multi-factorial disease which claims many young lives. There are very few Indian studies that have investigated antiphospholipid antibodies (APLs) in MI patients. APLs have been implicated in arterial thrombosis including premature coronary artery and cerebrovascular thrombosis. In the present study, the prevalence of two clinically significant APLs--anticardiolipin antibody (ACA) and lupus anticoagulants (LA) in young MI patients was studied and compared with age- and sex-matched controls. Fifty healthy blood donors and 40 young MI patients (less than 45 yrs) diagnosed according to the American Heart Association guidelines were recruited for the study. The criteria for diagnosis were presence of atleast two of three classical findings including: clinical symptoms, diagnostic ECG, and presence of one or more cardiac biomarkers out of raised CK-MB isoform and T-troponin on serial measurement. LA and ACA were tested by lupus-sensitive activated partial thromboplastin time (aPTT) and ELISA respectively. Elevation of ACA was observed in 9 patients, while 6 were positive for LA. ACA of IgG isotype was detected in 8 patients. One patient had LA and raised ACA of IgG and IgM isotypes. Antiphospholipid antibodies were found to be significantly associated with MI in young patients, when considered together (p < 0.05) and in coronary thrombosis, mild elevation of ACA may be considered significant.
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PMID:Antiphospholipid antibodies in young myocardial infarction patients. 1832 Aug 48

We report a case of acute myocardial infarction due to non-antiphospholipid-related coronary artery thrombosis as the presenting manifestation of systemic lupus erythematosus in a young patient. We present the acute workup and the results of successful transcatheter coronary intervention. The causes of acute myocardial infarction and coronary artery thrombosis in pediatric patients are reviewed.
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PMID:Acute myocardial infarction as the presenting symptom of systemic lupus erythematosus. 1938 19

Coronary thrombosis as a manifestation of the antiphospholipid syndrome is very uncommon. We report a 25 year-old male without known cardiovascular risk factors that suffered an acute myocardial infarction as the initial manifestation of the antiphospholipid syndrome. His coronary angiogram demonstrated a single thrombotic lesion in the anterior descending artery without coronary atheromatosis. Anticardiolipin, anti B2 Glycoprotein I antibodies, and lupus anticoagulant were all positive. Besides the usual management of the coronary thrombosis, the patient was treated with permanent oral anticoagulation. Three months later, a CT coronary angiogram showed complete reperfusion of the involved artery .
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PMID:[Acute myocardial infarction in a man without coronary atheromatosis and antiphospholipid syndrome: report of one case]. 2009 8


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