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)

A 48-year-old woman admitted with progressive dyspnea had previously been diagnosed with systemic lupus erythematosus, antiphospholipid syndrome, and chronic renal failure, and had undergone mitral valve replacement with a Carpentier-Edwards pericardial bioprosthesis for mitral insufficiency 9 years before. She suffered a cerebral infarction 5 years earlier, despite appropriate anticoagulant therapy. On admission, echocardiography showed severe bioprosthetic stenosis. Repeat mitral valve replacement was conducted using a Mosaic bioprosthesis. On postoperative day 2, when heparinization was commenced, she suddenly had an epileptic fit. She also developed ischemic necrosis of the fingers and toes, considered secondary to microthrombosis. Aspirin was administered and heparin replaced by warfarin sodium. Necrosis gradually disappeared, and she was discharged 3 months after surgery. The original bioprosthesis showed degenerative changes with significant thrombus formation on cusps, thought to be mainly due to her hypercoagulable state. Considering the thrombophilic tendency in patients with antiphospholipid syndrome, strict management of anticoagulant therapy is required.
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PMID:Secondary mitral valve replacement in antiphospholipid syndrome and chronic renal failure. 1180 97

The objective of this study was to study cardiac valve morphology and function and ventricular function in systemic lupus erythematosus (SLE) patients with and without co-existing cardiovascular disease (CVD) and in population controls. Twenty-six women (52 +/- 8.2 years) with SLE (SLE cases) and a history of CVD (angina pectoris, myocardial infarction, cerebral infarction or intermittent claudication) were compared with 26age-matched women with SLE but without manifest CVD (SLE controls) and 26 age-matched control women (population controls). Echocardiographywas performed to assess valvular abnormalities and manifestations of ischaemic heart disease. Thirteen of the 26 SLE cases but only one of the SLE controls and one of the population controls had cardiac valvular abnormalities. Three of the SLE cases had already undergone valve replacement and another had significant aortic insufficiency; the other nine had thickening of mainly mitral leaflets without hemodynamic significance. Among SLE cases, patients with valvular abnormalities had higher homocysteine (P < 0.001) and triglyceride (P = 0.02) concentrations than patients without valvular disease. In contrast atherosclerosis as determined by IMT, oxidized LDL as measured by the monoclonal antibody E06, autoantibodies against epitopes of OxLDL (aOxLDL) or phospholipids (aPL), disease duration or activity, or acute phase reactants did not differ between SLE cases with or without valvular abnormalities. Valvular abnormalities were not more common in SLE cases with stroke as compared to those with myocardial infarction, angina or claudication. In conclusion, valvular abnormalities are strongly associated with CVD in SLE. Raised levels of homocysteine and triglycerides characterize patients with cardiac valve abnormalities.
Lupus 2002
PMID:Cardiac valvular abnormalities are frequent in systemic lupus erythematosus patients with manifest arterial disease. 1247 5

Bone marrow transplantation is becoming a powerful strategy for the treatment of hematologic disorders (leukemia, aplastic anemia, etc.), congenital immunodeficiencies, metabolic disorders and also autoimmune diseases. Using various animal models for autoimmune diseases, we have previously found that allogeneic (not autologous) bone marrow transplantation can be used to treat autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, immune thrombocytic purpura, insulin-dependent diabetes mellitus, chronic glomerulonephritis and certain types of non-insulin-dependent diabetes mellitus. In contrast, we have found that the transplantation of T-cell-depleted bone marrow cells or partially purified hemopoietic stem cells from autoimmune-prone mice to normal mice leads to the induction of autoimmune diseases in the recipients. These findings have recently been confirmed even in humans; autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, multiple sclerosis and Crohn's disease were resolved after allogeneic bone marrow transplantation. However, there have recently been reports on the rapid recurrence or persistence of autoimmune diseases after autologous bone marrow transplantation. Conversely, the adoptive transfer of autoimmune diseases such as myasthenia gravis, insulin-dependent diabetes mellitus and Graves' disease by allogeneic bone marrow transplantation from donors to recipients has been reported. Owing to these findings, we have proposed that autoimmune diseases are "stem cell disorders." We have thus succeeded in treating autoimmune diseases in various autoimmune-prone mice, except MRL/lpr mice, by conventional bone marrow transplantation. The MRL/lpr mouse itself is radiosensitive (<8.5 Gy), while the abnormal hemopoietic stem cells of the MRL/lpr mouse are radioresistant (>8.5 Gy); conventional bone marrow transplantation (8.5 Gy plus bone marrow transplantation) has a transient effect on autoimmune diseases, which recur three months after the bone marrow transplantation. However, bone marrow transplantation plus bone grafts (to recruit donor stromal cells) completely prevents the recurrence of autoimmune diseases in MRL/lpr mice. Donor-derived stromal cells (including mesenchymal stem cells) thus seem to play a crucial role in successful allogeneic bone marrow transplantation, since there is a major histocompatibility complex restriction between hemopoietic stem cells and stromal cells. We have, however, found that the combination of bone marrow transplantation plus bone grafts has no effect on the treatment of autoimmune diseases in MRL/lpr mice, since MRL/lpr mice become more radiosensitive after the onset of lupus nephritis due to the development of uremic enterocolitis. To reduce the cytotoxic effect of radiation on the intestine, we carried out fractionated irradiation and devised a new strategy. We injected allogeneic whole bone marrow cells (including a small number [<3%] of T cells, hemopoietic stem cells and stromal cells) from donors directly into the intra-bone marrow of recipients so that donor-derived hemopoietic cells including stromal cells could effectively accumulate in the bone marrow. All the MRL/lpr mice survived more than one year (>60 weeks after birth) without the recurrence of autoimmune diseases, and immunological functions were completely restored even when the radiation dose was reduced to 5 Gy x 2. These findings suggest that intra-bone marrow injection-bone marrow transplantation can be used to treat intractable autoimmune diseases under reduced radiation doses without using any immunosuppressants.Intra-bone marrow injection-bone marrow transplantation seems to be the best strategy for allogeneic bone marrow transplantation: 1) no graft-versus-host disease develops even if T cells are not depleted from the bone marrow; 2) no graft failure occurs even if the dose of radiation as the conditioning for bone marrow transplantation is reduced to 5 Gy x 2; 3) hemopoietic recovery is rapid; and 4) T-cell functions are completely restored even in donor-recipient combinations across the major histocompatibility complex barriers. Using cynomolgus monkeys, we have recently established a new method (the "perfusion method") for collecting bone marrow cells from the long bones (femur, humerus, etc.) without peripheral blood contamination. This method has various advantages: 1) no graft-versus-host disease develops even in cynomolgus monkeys, since the percentage of T cells in the bone marrow cells collected is less than 3%; 2) a large number of bone marrow cells can be collected quickly and safely; and 3) the bone marrow cells collected contain stromal cells including mesenchymal stem cells. We therefore believe that this method (intra-bone marrow injection-bone marrow transplantation in conjunction with the perfusion method) will become a powerful new strategy for not only allogeneic bone marrow transplantation but also organ transplantation in conjunction with bone marrow transplantation. Furthermore, this method could become a valuable strategy in regeneration therapy for injured organs and tissues (myocardial infarction, cerebral infarction, Alzheimer's disease, etc.), since it can efficiently reconstitute the recipient with both donor-derived hemopoietic stem cells and mesenchymal stem cells.
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PMID:Bone marrow transplantation: a new strategy for intractable diseases. 1253 88

Neuropsychiatric (NP) manifestations in patients with systemic lupus erythematosus (SLE) [NPSLE] and prognostic factors were studied in 91 patients. There were 98 NP episodes, of which 78 (79.6%) occurred within the first year of the disease. Twenty-six patients (6.7%) had NPSLE as an initial presentation of the disease. There were seizures in 53 episodes (54.1%), psychosis in 13 (13.3%), acute confusion state in 11 (11.2%), abnormal consciousness in 6 (6.1%), transverse myelitis in 6 (6.1%), peripheral neuropathy in 5 (5.1%), cerebral infarction in 2 (2.0%) and aseptic meningitis in 2 (2.0%). Most forms of NPSLE responded well to high dose corticosteroids. Anti-convulsant therapy could be discontinued within a median duration of 3 months after the SLE activity was under control, and without significant recurrence of seizures. The 5-year and 10-year survival rates of patients with NPSLE were 75.9% and 50.6%, respectively. Patients with NPSLE had significantly more cutaneous vasculitis and less arthritis than those without.
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PMID:Neuropsychiatric manifestations in Thai patients with systemic lupus erythematosus. 1258 42

A number of previous studies have shown that anti-phospholipid(aPL) antibodies(Abs) do not bind primarily to the negatively-charged phospholipid itself but rather to complexes of the phospholipid and plasma proteins, and that the most common antigenic targets are beta 2-glycoprotein I recognized by anticardiolipin Abs and prothrombin recognized by most lupus anticoagulants. However, resent studies suggest that other phospholipid-binding proteins, particularly protein C, protein S, and annexin V, may be important targets as well. To clarify the association between the various types of aPL Abs and thrombotic complications in patients with systemic lupus erythematosus(SLE), we examined the prevalence of aPL Abs to various phospholipid-binding proteins(beta 2-glycoprotein I, prothrombin, protein C, protein S, and annexin V). We found that anti-beta 2-glycoprotein I Abs may be associated primarily with cerebral infarction and femoral artery thrombosis, and that anti-protein S Abs may be associated primarily with venous thromboembolism and renal thrombotic microangiopathy. Furthermore, anti-annexin V Abs might be closely related to fetal loss. These findings suggest that thrombotic complications in SLE depend on the antigenic specificities of aPL Abs, alone or in combination.
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PMID:[Association between anti-phospholipid antibodies and thrombotic complications in systemic lupus erythematosus]. 1270 97

Antibodies to beta2-glycoprotein I (anti-beta2GPI) have been associated with recurrent thrombosis and pregnancy morbidity. However, the prevalence of anti-beta2GPI in children suffering from cerebral infarction is unknown. We report on a 20-month-old boy who had an ischemic stroke, secondary to antiphospholipid syndrome with high titers of immunoglobulin G anti-beta2GPI (first titer: 132 U; second titer 6 weeks later: 350 U; normal range: 0-100 U). Anticardiolipin antibodies and lupus anticoagulant tests were negative. All other causes of infarction were excluded. Laboratory studies showed anti-beta2GPI IgG levels of 164 U and 216 U at 6 months and 2 years, respectively, after the onset. The patient received treatment with low-dose aspirin. To our knowledge, this is the first reported case of childhood ischemic stroke with only anti-beta2GPI but no antibodies detectable in standard antiphospholipid assays. This case supports the recommendation of others to search for these antibodies in the presence of strong clinical suspicion of antiphospholipid syndrome, when anticardiolipin antibodies and lupus anticoagulant tests are negative.
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PMID:Anti-beta2-glycoprotein I antibodies and ischemic stroke in a 20-month-old boy. 1283 87

We report on a 13-year-old female with systemic lupus erythematosus (SLE) who exhibited symptoms of severe migraine and familial moyamoya disease. Cerebral magnetic resonance angiography (MRA) showed stenosis and occlusion of the bilateral internal carotid arteries associated with the development of collateral circulation (moyamoya vessels). In a child, as in this case, headaches with cerebral infarction associated with moyamoya disease are unusual. Few cases of SLE associated with familial moyamoya disease have been reported, with no previous reports of such cases from Korea. There were no evidences of antiphospholipid syndrome, and activity of SLE or other risk factors for cerebral occlusion were also absent.
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PMID:Systemic lupus erythematosus associated with familial moyamoya disease. 1471 35

Although central nervous system involvement is an important manifestation of systemic lupus erythematosus (SLE), chorea is a relatively uncommon complication. A strong association between chorea and the presence of antiphospholipid antibodies (aPLs) has been reported in patients with SLE, lupus-like disease, or primary antiphospholipid syndrome. We describe a patient with lupus nephritis and cerebral infarction, who subsequently developed recurrent hemichorea associated with increased aPLs levels. A 7-year-old boy suffered from lupus nephritis and a left middle cerebral artery infarction associated with aPLs. He subsequently experienced two episodes of right hemichorea associated with increased aPLs levels without any evidence of further neurological lesions by brain computed tomography or magnetic resonance imaging. The previous left cerebral artery infarction might have increased the susceptibility of the left basal ganglia to the effects of aPLs that contributed to the development of the right hemichorea in this patient.
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PMID:Hemichorea with antiphospholipid antibodies in a patient with lupus nephritis. 1474 Feb 86

Dementia is a very rare neurological manifestation of systemic lupus erythematosus (SLE) and has a deep link with antiphospholipid antibodies (APL) and cerebral infarction in its development. However, nonvascular dementia irrelevant to APL or cerebral infarction has not been reported in patients with SLE until now. We describe a case of reversible dementia in an SLE patient without APL or cerebral infarction which was successfully treated with corticosteroid and cyclophosphamide. There are two significant points in this case. One is that humoral factors other than APL might be involved in the development of dementia. Secondly, reversible dementia without APL or cerebral infarction may respond more favorably to immunosuppressive therapy.
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PMID:Reversible dementia in systemic lupus erythematosus without antiphospholipid antibodies or cerebral infarction. 1499 39

A 38-year old female with underlying systemic lupus erythematosus was admitted with tuberculous meningoencephalitis. After an initial good response to anti-tuberculous treatment, she developed cerebral infarction and profound hyponatremia. This was due to cerebral salt wasting syndrome, which has only previously been described in 2 cases. The difficulties in diagnosis and management of this case are discussed.
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PMID:Cerebral infarction and cerebral salt wasting syndrome in a patient with tuberculous meningoencephalitis. 1511 43


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