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)

We report a 55-year-old female patient with antiphospholipid syndrome secondary to systemic lupus erythematosus. The patient had undergone coronary artery bypass grafting for myocardial infarction due to left main trunk stenosis at the age of 52. Subsequently, she developed aortic insufficiency and underwent aortic valve replacement without any hemodynamic or hemostatic problems. Both coronary and valve disease should be considered in patients with antiphospholipid syndrome secondary to systemic lupus erythematosus.
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PMID:Aortic valve replacement after previous coronary artery bypass grafting in a patient with antiphospholipid syndrome. 958 74

In a subset of systemic lupus erythematosus (SLE) patients, antiphospholipid syndrome, characterized by occurrence of anti-cardiolipin (CL) antibodies, thrombocytopenia, thrombosis and recurrent intrauterine fetal death occurs. Male (NZW x BXSB)F1 mice, carrying the BXSB Yaa gene, serve as a model for SLE-associated antiphospholipid syndrome. Using microsatellite markers in the NZW x (NZW x BXSB)F1 backcross male progeny, we mapped BXSB alleles contributing to the generation of anti-CL antibodies, platelet-binding antibodies, thrombocytopenia and myocardial infarction. Generation of each disease character was controlled by two major independently segregating dominant alleles, i.e. those on chromosomes (Chr.) 4 and 17 for anti-CL antibodies, Chr. 8 and 17 for both anti-platelet antibodies and thrombocytopenia and, to our surprise, Chr. 7 and 14 for myocardial infarction, and that a combination of the two alleles appeared to produce full expression of each character, as a complementary gene action. The alleles on Chr. 17 linked to the above three characters were all mapped in close proximity to the H-2 complex. Therefore, no single factor such as anti-CL antibodies can explain the pathogenesis of SLE-associated antiphospholipid syndrome. Rather, a combination of susceptibility alleles such as described here, along with additional modifying loci, i.e. BXSB Yaa and some from NZW, characterizes unique SLE features in male (NZW x BXSB) F1 mice. There are potentially important candidate genes which may be linked to the syndrome.
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PMID:Multigenic control of lupus-associated antiphospholipid syndrome in a model of (NZW x BXSB) F1 mice. 975 57

One hundred and seventy patients with rheumatological disease diagnosed before their 16th birthday and still on follow up were studied retrospectively. They were seen within the last 3 years at KK Women's and Children's Hospital, Tan Tock Seng Hospital, National Skin Centre or Singapore General Hospital. Of these, 89 were still less than 16 years old at the time of study. The majority had systemic lupus erythematosus (51.8%). Many were on long-term follow-up for persistent disease, including renal manifestations (47.7%), neurological manifestations (26.1%) and haemolytic anaemia (15.9%). Photosensitivity and malar rash were more common than in Western studies while arthritis was less common. Anti-phospholipid antibodies were found in children complicated by myocardial infarction, pulmonary hypertension, Raynaud's phenomenon, cerebral and gut lupus. Children with juvenile chronic arthritis comprised 28.8% and juvenile dermatomyositis 10%. The male predominance and lack of uveitis in children with pauciarticular JCA were striking. Rarer conditions included polyarteritis nodosa, scleroderma, rheumatic fever with arthritis, polychondritis and Behcet's disease. Many diseases may first present with a rheumatological complaint. This review of features of local children highlights similarities and differences with Western data. It also provides information for planning long-term care, multidisciplinary clinics, group physiotherapy sessions, educational programmes and support groups.
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PMID:One hundred and seventy cases of childhood-onset rheumatological disease in Singapore. 979 53

In prospective studies, increased levels of cardiolipin-biding antibodies and autoantibodies to oxidized low-density lipoprotein (LDL) have been observed in patients with myocardial infarction (MI). These findings suggest that antiphospholipid antibodies may contribute to the development of MI. The 'oxidative-modification hypothesis' in the pathogenesis of atherosclerotic heart disease is based on the oxidation of LDL, its accumulation into arterial wall, and the development of chronic inflammation in the atheroma. Evidence of enhanced lipid peroxidation and its association with antiphospholipid antibodies has been recently reported in SLE patients. There is also epidemiological data showing a remarkably increased risk of MI in SLE. In this review, the role of different types of antiphospholipid antibodies in the development of atherosclerotic heart disease is evaluated with particular attention to their potential pathogenic mechanisms and the possibilities in the prevention of MI associated with antiphospholipid antibodies.
Lupus 1998
PMID:Antiphospholipid antibodies and myocardial infarction. 981 90

Women with inherited or acquired thrombophilia are at increased risk for venous thromboembolism (VTE) when they use oral contraceptives (OCs) of either the second or third generation. For women who are heterozygous for Factor V Leiden, the risk is probably 28 to 50 of 10,000 women-years compared to 2 to 5 of 10,000 years for those not known to have thrombophilia. The thrombotic risk is highest during the first year that OCs are used. Whether women with thrombophilia are at increased risk for VTE when they use hormone replacement therapy (HRT) has not been assessed in any study. For women without thrombophilia, the risk for VTE associated with HRT is probably 2 to 3 of 10,000 years. The benefits of HRT include reduced risk for myocardial infarction and Alzheimer's disease, and increased bone density. The physiological changes induced by HRT are not the same as those induced by OCs. Small studies have suggested that for women who have additional risks of thrombosis (i.e., perioperative setting, underlying systemic lupus erythematosus), HRT does not confer the same increased risk of thrombosis, as does the use of OCs. Until data are available to address the magnitude of any increase in thrombotic risk induced by HRT for women with thrombophilia, physicians probably serve their patients best by providing information about the benefits of HRT, emphasizing that the risk of VTE is unknown, and encouraging patients to take an active role in decisions about their healthcare.
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PMID:Women with thrombophilia: assessing the risks for thrombosis with oral contraceptives or hormone replacement therapy. 984 Jun 90

The authors present the cases of two young patients, a man and a woman, who presented with myocardial infarction, in the absence of ischemic heart disease or stenosis of the coronary arteries. The woman was known to have systemic lupus erythematosus (SLE) for the past 3 years (the immunoglobulin M [IgM] anticardiolipins antibodies were positive), without a history of coronary risk factors. Suddenly she presented with acute chest pain on rest that lasted 4 hours and culminated in anterior wall myocardial infarction. She was admitted to the coronary care unit, where no thrombolysis was given. She did not have echocardiographic evidence of Libman-Sacks endocarditis, but myocardial infarction was evident at the electrocardiogram (ECG). The young man had SLE (the IgM anticardiolipins were absent, but he was positive for lupus anticoagulant antibodies), he was hyperlipidemic, was a moderate smoker and moderately obese, and had no history of ischemic heart disease. He suddenly presented with an acute myocardial infarction documented by ECG, enzymes, and gammagraphy. In both patients, coronary angiography findings were normal and myocardial biopsy did not show evidence of arteritis. The relevance of these cases is the rare association of ischemic heart disease in SLE, with normal coronary arteries and without evidence of arteritis or verrucous endocarditis.
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PMID:Myocardial infarction in patients with systemic lupus erythematosus with normal findings from coronary arteriography and without coronary vasculitis--case reports. 1008 5

BACKGROUND: Thrombophilia may be associated with premature atherosclerosis, an increased susceptibility to primary arterial thrombosis and an increased failure rate for peripheral vascular or endovascular interventions. The aim of this study was to determine the prevalence of thrombophilia in patients with intermittent claudication (IC). METHODS: This was a prospective study of 116 consecutive new patients (70 men; median age 65 (range 43-84) years) referred to this regional vascular surgery unit with IC. Patients on warfarin, or who had previously undergone lower limb reconstruction and/or angioplasty, were excluded. RESULTS: Thrombophilia was demonstrated in 24 patients (21 per cent). The commonest abnormality (15 patients, 13 per cent) was a raised level of anticardiolipin antibody (ACLA) (11 immunoglobulin (Ig) M, four IgG). Other abnormalities comprised: lupus anticoagulant (one), protein C deficiency (two), protein S deficiency (two), activated protein C resistance (one) and factor V Leiden heterozygosity (three). All abnormalities were confirmed on repeat testing. No patient had a history of venous thrombosis. There was no statistically significant relationship between ACLA status and age, sex, ankle : brachial pressure index, previous myocardial infarction or stroke, previous carotid endarterectomy or coronary artery surgery, serum cholesterol, current use of antiplatelet agents or current smoking status. CONCLUSION: Almost one-quarter of new patients referred to this regional vascular unit with IC have thrombophilia; over half of those affected have a raised ACLA level compatible with the antiphospholipid syndrome. At present, the clinical significance and management implications of these abnormalities remain unknown.
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PMID:Vascular surgical society of great britain and ireland: prevalence and significance of thrombophilia in patients with intermittent claudication 1036 36

Although the short- and medium-term (5-10 years) outcome of patients with lupus nephritis has been studied extensively, there are very few data on the second and subsequent decades. We studied outcome in 110 local patients investigated at a single centre before 1986, who all had potential follow-up of more than 10 years (actual 2-31 years, median 15.5 years). At last follow-up, 40 patients were dead and 70 alive, nine of whom were on maintenance dialysis or transplanted, actuarial survivals being 84%, 72%, 62%, 61% and 54% at 5, 10, 15, 20 and 25 years for the group as a whole. Survival was better in the cohort 1976-86 (n = 60) than in that from 1963-75 (n = 50) (90, 81 and 76% vs. 78, 56 and 43% at 5, 10 and 15 years, p < 0.001). Sepsis (12) and myocardial infarction (8) were the principal causes of death. Of living patients with renal function, 38% had normal urine and renal function, 11 were off all treatment (19%), 62% had persistent proteinuria and 18% had reduced but generally stable renal function. Renal failure, in those patients who developed it, occurred during the first decade and none of 67 patients actually followed more than 10 years subsequently went into renal failure. Induction treatment was with prednisolone, combined with azathioprine in more severe forms of nephritis, and from the middle 1970s to 1986, 30 with methylprednisolone and in 12 cases plasma exchange. Seventeen other patients were treated using oral cyclophosphamide during the 1960s. No patient received i.v. cyclophosphamide as induction therapy, although nine patients had this form of treatment later, largely because of non-compliance. Serious complications of lupus and/or its treatment occurred in 49%: sepsis in 32, ischaemic heart disease in 20, thrombosis in one and avascular necrosis of bone in eight. In contrast, fracturing osteoporosis occurred in only three, and cataracts requiring surgery and diabetes mellitus in none. The very long-term outlook of lupus nephritis, especially its more severe forms, has improved, but that with current management strategies only a minority of patients are able to stop treatment altogether, and the incidence of serious complications is high.
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PMID:The very long-term prognosis and complications of lupus nephritis and its treatment. 1039 9

Observational cohort studies in SLE have led to the description of accelerated atherosclerosis as an important cause of mortality and morbidity in this disease. The clinical observation of coronary artery disease occurring in premenopausal females with SLE gave rise to the concept of the bimodal mortality pattern. This pattern was confirmed in autopsy and epidemiological studies. These studies identified hypercholesterolemia and particularly its persistence in the first three years of disease, hypertension, and lupus itself as important risk factors for the development of accelerated atherosclerosis in these patients. It also became evident that corticosteroid therapy plays an important role in the elevation of plasma lipids while antimalarials resulted in a reduction of plasma cholesterol, LDL, and VLDL, especially in steroid-induced hyperlipidemia. Studies of clinical outcomes for atherosclerotic disease (angina, myocardial infarction) have shown a prevalence of 6-12% in a number of SLE cohorts. However, more sensitive investigations including myocardial perfusion imaging and carotid ultrasound have demonstrated a prevalence of atherosclerotic disease in 40% of patients studied. Further studies of SLE disease process, including immunological factors, may more clearly define the pathogenesis of accelerated atherosclerosis in patients with SLE, and may help elucidate mechanisms of atherosclerosis in the general population.
Lupus 2000
PMID:Accelerated atheroma in lupus--background. 1080 81

Coronary artery disease (CAD) is a major cause of morbidity and mortality in SLE, including the Hopkins Lupus Cohort. Currently, 9% of the cohort have had clinical evidence (angina or myocardial infarction) of CAD. In our initial prospective study we found that duration of prednisone, hypertension, hyperlipidemia and obesity were risk factors for later CAD. We can now extend that list to include age, male sex, elevated homocysteine, renal insufficiency and antiphospholipid antibodies. Many of the risk factors are amenable to intervention, but the timing of intervention, and the effectiveness of intervention, must be determined.
Lupus 2000
PMID:Detection of coronary artery disease and the role of traditional risk factors in the Hopkins Lupus Cohort. 1080 83


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