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 antibody syndrome (APS) is defined broadly by the presence of antiphospholipid antibodies, venous and arterial thrombosis, thrombocytopenia and fetal wastage. APS can be primary or secondary, in which APS occurs in the context of another defined disease such as autoimmune disease, malignancy, drug-induced disease, etc. APS is primary in one-half of patients and secondary in the rest, mainly to systemic lupus erythematosus. Several cardiac manifestations of APS have been reported. These include valvular heart disease, coronary artery disease, intracardiac thrombosis and cardiomyopathy. The literature has shown a prevalence of approximately 35% of valvular abnormalities detected by echocardiography in patients with APS. A patient with primary APS who developed aortic stenosis with vegetations on a bioprosthetic porcine valve is presented.
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PMID:Antiphospholipid antibody syndrome with involvement of a bioprosthetic heart valve. 970 81

We report a case of 52-year-old woman with primary antiphospholipid syndrome who developed mitral insufficiency and chronic renal failure. Continuous ambulatory peritoneal dialysis was started preoperatively due to thrombocytopenia that was aggravated by hemodialysis. Mitral annuloplasty was performed since the mitral valve was not severely damaged. Her postoperative hemodynamics were stable, and anticoagulant therapy was controlled easily. She recovered from severe thrombocytopenia while on continuous ambulatory peritoneal dialysis. Valvular heart disease is a well known feature of primary antiphospholipid syndrome, and there have been several reports about valve replacement in patients who had antiphospholipid syndrome with or without systemic lupus erythematosus. However, valve repair has been reported in only a few such patients. We believe that valve repair is better than valve replacement in patients with antiphospholipid syndrome because of its hypercoagulable tendency. In addition, it seems that continuous ambulatory peritoneal dialysis is a suitable method for the perioperative management of patients with antiphospholipid syndrome who suffer from chronic renal failure as well as thrombocytopenia, and require cardiac surgery under cardiopulmonary bypass.
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PMID:Mitral insufficiency associated with primary antiphospholipid syndrome and chronic renal failure. 1130 57

Severe mitral valve regurgitation due to systemic lupus erythematosus is a rare cause of valvular heart disease, necessitating valve surgery. Currently, there are 36 case reports in the world medical literature of mitral valve replacement or repair in patients who have lupus. The current trend in mitral valve surgery is toward anatomic valve repair. In patients who have systemic lupus erythematosus, however, valve repair often leads to repeat surgery and valve replacement. We report the cases of 5 patients with lupus and severe mitral valve regurgitation who underwent mitral valve surgery. In 3 of these patients, replacement with a mechanical prosthetic mitral valve was performed with good long-term results. In the other 2 patients, mitral valve repair was performed, but only 1 of the repairs was successful. The 2nd patient required subsequent replacement with a mechanical valve. To our knowledge, this report of 5 patients is the largest series of mitral valve surgery in patients with lupus. These results, along with a review of the literature, suggest the superiority of mechanical prosthetic valve replacement to repair in patients who have systemic lupus erythematosus.
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PMID:Mitral valve replacement and repair. Report of 5 patients with systemic lupus erythematosus. 1133 Jul 42

BACKGROUND: Valvular heart disease is the most important cardiac manifestation of systemic lupus erythematosus (SLE). We performed a study to determine the presence of valvular heart disease in our patients with SLE. METHODS: We performed clinical, electrocardiographic, transthoracic echocardiographic and laboratory evaluations in 24 patients with SLE. The echocardiographic findings were compared with those of 10 age- and sex-matched volunteers. RESULTS: None of the 24 patients had obvious symptoms of cardiac origin. Valvular abnormalities were common. Valvular thickening was the most predominant finding (more than 50%), followed by mitral valvular regurgitation (12.5%) and pericardial effusions (12.5%). Valvular abnormalities were uncommon in the control group. CONCLUSION: Valvular heart disease is common in our patient population with SLE, although haemodynamically significant valvular dysfunction is rare.
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PMID:Valvular heart disease associated with systemic lupus erythematosus - the Tygerberg Hospital experience. 1144 75

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

Primary prevention of strokes in patients with antiphospholipid antibodies (APLs) with or without systemic lupus erythematosus (SLE) is not well known. The same applies to patients with SLE and valvular heart disease. The decision should be made on an individual basis until further studies become available. Special consideration for preventive antithrombotic treatment should be given to patients with persistent high titers of immunoglobulin G APLs, which require protein cofactor for detection, presence of lupus anticoagulant, or left-sided cardiac valve lesions. High-level oral anticoagulation with warfarin is still the preferred treatment for secondary prevention of strokes in patients with antiphospholipid antibody syndrome (APS) with or without SLE or with cardiac valvular lesions. Immunosuppression should only be used in patients with active SLE disease. There is no evidence so far to support its use in patients with primary APS. Advances in identifying unique APL features that are associated with increased risk for thrombosis will hopefully allow a more rational treatment for primary and secondary prevention of strokes in these patients in the near future.
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PMID:Stroke in Patients with Systemic Lupus Erythematosus and Antiphospholipid Antibody Syndrome. 1509 14

Thirty-seven patients with systemic lupus erythematosus underwent complete clinical and laboratory evaluations, including antiphospholipid antibodies and lupus anticoagulant, magnetic resonance imaging of the brain, and transesophageal echocardiography. Cerebrovascular disease manifested as stroke, transient ischemic attack, or cerebral infarcts in patients with nonfocal neurologic deficits was detected in 19 patients (51%), and significant left-sided valvular heart disease in 25 (68%). Valve vegetations, valve thickening, valve regurgitation, and lupus anticoagulant antibody occurred 2 to 3 times more often in patients with than without cerebrovascular disease (all p < or =0.04) and were the only independent predictors of cerebrovascular disease (odd ratios 5.3 to 10.6, all p < or =0.03). Thus, valvular heart disease probably exacerbated by hypercoagulability appears to be a source of embolic ischemic brain injury and cerebrovascular disease.
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PMID:Valvular heart disease as a cause of cerebrovascular disease in patients with systemic lupus erythematosus. 1595 May 67

Antiphospholipid syndrome spans many medical disciplines. Classic criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical complications of thrombosis or pregnancy loss. Other common associated manifestations include livedo reticularis, thrombocytopenia, valvular heart disease, and nephropathy with renal insufficiency, hypertension, and proteinuria. Treatment of serious complications with anticoagulation is standard; generally warfarin for thrombosis and aspirin/heparin for pregnancy prophylaxis. Detailed recommendations regarding precise intensity and duration of anticoagulation are still a subject of debate.
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PMID:Antiphospholipid syndrome: review. 1600 69

The valvular heart disease in systemic lupus erythematosus (SLE) is associated with substantial morbidity and mortality. Current therapy includes symptomatic measures and valve replacement. Overall mortality of valve replacement has been reported to be as high as 25%. Most cases of Libman-Sacks endocarditis in the literature reported dominant aortic regurgitation. We present this unusual case of a young female patient with SLE and glomerulonephritis warranting emergency isolated aortic valve replacement (AVR) for severe calcific aortic stenosis. The literature is reviewed with specific focus on the pathogenesis of and acute treatment options for this extremely rare occurrence.
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PMID:Emergency aortic valve replacement in systemic lupus erythematosus. 1681 55

The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterised by autoantibody production and vascular thrombosis or pregnancy morbidity. Autoantibodies generated against phospholipid and phospholipid-binding proteins often impair phospholipid-dependent clotting assays (lupus anticoagulants). These autoantibodies activate endothelial cells, platelets and biochemical cascades and can exist in autoimmune disorders such as lupus. Consistently positive antibodies may worsen the severity of thrombo-occlusive disease. The most common neurological manifestations of APS include stroke and transient ischaemic attacks due to arterial thromboses. Antiphospholipid antibodies may cause additional neurological impairments through both vascular and immune mechanisms. Antiaggregant or anticoagulant therapies are indicated for APS-related ischaemic strokes. Treatment regimens for asymptomatic antibody-positive patients and those with refractory disease remain controversial. There is scant literature on neurological APS manifestations in paediatric patients. Assessment of traditional cardiovascular and inherited thrombophilia risk factors is essential in patients with APS. Modifiable risk factors and valvular heart disease may worsen thrombotic and cerebrovascular outcomes. Alternative therapies such as statins, anti-malarials, angiotensin-converting enzyme inhibitors and thrombin inhibitors warrant further research.
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PMID:Neurological manifestations of the antiphospholipid syndrome: risk assessments and evidence-based medicine. 1759 87


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