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 case of a 27-year-old Japanese woman with type A acute aortic dissection who had been diagnosed with systemic lupus erythematosus (SLE) is presented. The patient also had aortic regurgitation due to non-infective endocarditis and systemic hypertension, and had been maintained on steroid therapy for 15 years. Her twin sister was also diagnosed with SLE. The patient was admitted to emergency due to severe back pain. A chest x-ray showed enlargement of the upper mediastinum. Echocardiography revealed a thickened and deformed aortic valve with aortic regurgitation and dissection of the ascending aorta, but pericardial effusion was not found. Computed tomography demonstrated aortic dissection extending from the ascending aorta to the abdominal aorta. Graft replacement of the ascending aorta and proximal aortic arch was performed under hypothermic circulatory arrest with retrograde cerebral perfusion. The patient recovered uneventfully. Aortic dissection complicated with SLE is extremely rare, and this is only the 15th case reported in the English or Japanese literature.
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PMID:Acute type A aortic dissection in a patient with systemic lupus erythematosus. 1245 14

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

Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.
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PMID:Heart disease in systemic lupus erythematosus: diagnosis and management. 1522 37

Cardiovascular involvements in antiphospholipid antibody syndrome have been recognized as a major complication of this disease. Furthermore, some papers report bioprosthetic heart valve also seems to be affected. A 32-year-old female with aortic regurgitation presented to our hospital. Further examination revealed high titer of anticardiolipin beta 2 glycoprotein 1 antibody, and she was diagnosed as having primary antiphospholipid antibody syndrome since the patient failed to match the criteria of systemic lupus erythematosus. Cardiopulmonary bypass was uneventfully conducted under systemic heparinization of usual dosage. Administration of warfarin sodium was started on the third postoperative day, and international normalized ratio was controlled from 2.0 to 2.5. On echocardiographic examination at 1 month, mean systolic gradient was 17 mmHg. Although transesophageal echocardiography at 2 years after surgery revealed no sign of valvular destruction or sclerosis, transaortic gradient had increased to 26 mmHg. Bioprosthetic stenosis was suspected probably due to pannus formation and the patient may have to undergo another valve replacement in the near future.
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PMID:Does antiphospholipid antibody syndrome affect bioprosthetic heart valve? Midterm echocardiographic report. 1572

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

Cardiac manifestations of antiphospholipid antibody syndrome (APLS) comprise a major complication. Herein we report our surgical treatment of aortic regurgitation in a patient with APLS. A 61-year-old woman was referred to our hospital with symptoms of congestive heart failure. Systemic lupus erythematosus had been diagnosed at the age of 36, and immunosuppressive therapy has been continuously performed. APLS was also diagnosed at the age of 55, after which cardiomegaly was noted on chest radiographs and aortic regurgitation was evident on echocardiography. Although immunosuppressive therapy had been continued, cardiac symptoms began to develop. With a presumed diagnosis of valvular disease associated with autoimmune disease, the aortic valve was replaced with a bioprosthesis. Noninfective endocarditis was confirmed in the excised specimen and was likely involved in APLS. The patient was discharged on postoperative day 26 without complications.
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PMID:Aortic valve replacement for aortic regurgitation in a patient with antiphospholipid antibody syndrome. 1767 59

A 13-year-old girl presented to our emergency with a one week history of fever and skin rash and new onset of chorea for the last three days. There was a long standing history of right predominant headache; followed by personality change, fatigue, arthralgia and weight loss over the last few months. Previous investigations by head CT and ophthalmological examination did not explain the symptoms. Further investigations revealed peri- and pancarditis with aortic insufficiency, a renal involvement with elevated creatinin, protein- and hematuria and a hemolytic anemia. Diagnosis of lupus eythematodes was confirmed by high ANA, anti-dsDNS and Anticardiolipin antibodies. Within the first 48 hours after admission there was significant deterioration with reduced vigilance and dysarthria. MRI of the brain and dopplersonography of cerebral vessels showed a complete thrombosis of the right medial cerebral artery with a small net of collaterals, irregularities of the left cerebral artery due to vasculitis and several subacute leftsided ischemias. Immunosuppressive therapy with high-dose corticosteroids and cyclophosphamid together with antithrombotic therapy induced an improvement of neurologic, renal and cardiac function.
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PMID:[Vasculitis as a reason of chronic headache]. 1854 40

A 31-year-old man with systemic lupus erythematosus had received long term steroid therapy. He had no compliant, but magnetic resonance imaging showed aortic dissection and annulo-aortic ectasia. Echocardiogram showed severe aortic regurgitation. Therefore aortic root replacement was performed. A histological study of the aortic wall demonstrated myxomatous degeneration in the media. He recovered uneventfully, except for receiving continious hemodia filtration during 4 days after the operation
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PMID:[Aortic dissection and annulo-aortic ectasia complicating systemic lupus erythematousus; report of a case]. 1952 12

Cardiac involvement is a recognized complication of systemic lupus erythematosus (SLE), which can involve most cardiac components, including pericardium, conduction system, myocardium, heart valves, and coronaries. Libman-Sacks (verrucous) endocarditis is the characteristic cardiac valvular manifestation. We report a patient with SLE who had severe aortic regurgitation caused by Libman-Sacks endocarditis. The patient underwent successful mechanical aortic valve replacement.
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PMID:Aortic valve replacement for Libman-Sacks endocarditis. 1963 41

A 37-year-old man was admitted to our hospital for precordial chest pain. He had taken prednisolone (5 mg/day) for systemic lupus erythematosus (SLE) and had been symptom free for the past 12 years. Echocardiography and contrast-enhanced CT of chest showed an enlarged ascending aortic aneurysm, which is rarely seen in SLE. Severe aortic regurgitation was also present, and surgical replacement of the ascending aorta and aortic valve was successfully accomplished by the Bentall procedure. Medial cystic necrosis in the ascending aorta, which is rarely seen in SLE angiopathy, was confirmed by histology. There were no significant histopathological findings in the aortic valve.
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PMID:Aortic aneurysm with severe aortic regurgitation in a patient with systemic lupus erythematosus. 2096 47


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