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 37-year-old man with systemic lupus erythematosus, who underwent an aortic valve replacement with a Carpentier-Edwards porcine valve for severe aortic insufficiency, was admitted to the hospital with pulmonary edema. Transesophageal echocardiography revealed severe aortic insufficiency arising from partial dehiscence of the valve sewing ring, as well as centrally from the valve cusp. In addition, marked thickening of the mitral valve was observed with severe eccentric regurgitation. At surgery, valvulitis of the native mitral and bioprosthetic aortic valves was demonstrated, with a perforation of the porcine valve cusp. After replacement of both valves, the patient had a stormy postoperative course with recurrent communications between the left ventricle and atrium requiring multiple surgeries and eventually died. This case illustrates the severity of valvulopathy and ensuing complications that can affect patients with systemic lupus erythematosus and demonstrates that the valvulopathy can affect bioprosthetic valves, a finding that has significant implications as to the type of valve replacement in these patients.
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PMID:Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. 867 31

A 40-year-old female had a history of fever, arthralgia, proteinuria, and dyspnea on effort twenty years ago, and was diagnosed as SLE, renal failure, and aortic regurgitation. She also suffered from pyelonephritis and sepsis due to the infection of E. coli. Preoperative examination revealed non-active phase of SLE. Echocardiography and aortography showed massive aortic regurgitation and operation was recommended. Operative findings showed fresh vegetation on the aortic leaflets, and aortic valve replacement (Tekna-Edwards 19 mm) was performed. Histological findings of the vegetation showed Libman-Sacks endocarditis and infectious endocarditis. Predonisolone was infused intravenously to prevent the acute deterioration of SLE after the operation. She was discharged from the hospital three weeks after the operation.
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PMID:[Aortic valve replacement due to Libman-Sacks endocarditis combined with infectious endocarditis]. 882 83

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

Two dimensional echocardiography with doppler examination was performed in 54 patients with systemic lupus erythematosus (SLE). Nine (17%) had significant cardiac involvement (four left ventricular hypertrophy, one moderate pericardial effusion, one severe aortic regurgitation, and three ventricular systolic dysfunction). We further studied diastolic function in 45 patients who did not have a major abnormality in echo. SLE was graded as active in 16 patients (SLEDAI > 5) and inactive in 29 patients. Twenty age- and sex-matched subjects acted as controls. The data were compared using one way ANOVA test. Patients with active disease had significant diastolic dysfunction compared to inactive patients and controls as indicated by increased peak A (P < 0.01) and decreased E/A ratio (P < 0.01). There was no linear correlation between disease activity and diastolic dysfunction if SLEDAI was considered as a continuous variable (r=0.29 for E/A). Anticardiolipin antibodies (both IgG and IgM) were elevated in five patients (13 studied). One of them had severe mitral regurgitation, one had trace mitral and aortic regurgitation and one had diastolic dysfunction. We conclude that asymptomatic diastolic dysfunction is present in SLE patients.
Lupus 1998
PMID:Echocardiography in systemic lupus erythematosus. 1034 21

Valvular involvement in patients with systemic lupus erythematosus (SLE) is not uncommon but patients rarely present with it. The mitral valve is most commonly involved. We report a 36-year-old man who had an episode of acute fever, arthritis, and acute aortic insufficiency with a small vegetation at the tip of the aortic valve mimicking infective endocarditis, proven later to be due to SLE. SLE should be considered as one of the uncommon causes of acute aortic insufficiency.
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PMID:Acute aortic valvulitis as an initial presentation of systemic lupus erythematosus. 1046 48

A 51-year-old woman with overt congestive heart failure with pleural and pericardial effusion was treated with furosemide and nifedipine, leading to improvement in her condition and a decrease in effusions. An echocardiography demonstrated mitral and aortic regurgitation with mitral valve prolapse, which caused the congestive heart failure. Since leukocytopenia and lymphocytopenia with arthralgia could be observed, serological investigations were performed. She was diagnosed as having systemic lupus erythematosus (SLE) with antiphospholipid syndrome, and started on a treatment of prednisolone and aspirin. Based on the treatment, the pleural and pericardial effusion went into complete remission, indicating that the serositis related to SLE had overlapped the heart failure. Since there was no evidence of any other diseases that could be responsible for the valvular lesions, we concluded that they were due to antiphospholipid syndrome. The administration of prednisolone had no significant effect on valvular morphology or function as demonstrated by echocardiography. When patients with valvular disease are seen, a valvulopathy related to antiphospholipid syndrome should be considered as part of the differential diagnosis.
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PMID:Overt congestive heart failure with mitral and aortic regurgitation due to antiphospholipid syndrome in a patient with systemic lupus erythematosus. 1085 59

Sensorineural hearing loss may occur in SLE, but aortic insufficiency has been very rarely reported. We are describing two patients with well-established SLE who developed bilateral hearing loss and aortic insufficiency, associated with serological evidence of active lupus. Neither patient had evidence of keratitis, and thus did not satisfy criteria for Cogan's syndrome. The aortic insufficiency in one patient stabilized after treatment with high doses of steroids while in the second patient, who refused medical treatment, it progressed requiring surgical valve replacement. Our observations suggest that the aortic valve and the inner ear may share some antigenic crossreactivity not shared by the cornea. In SLE patients, with sensorineural hearing loss, echocardiography should be performed looking for evidence of aortic insufficiency, which may be steroid responsive.
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PMID:Sensorineural hearing loss in conjunction with aortic insufficiency in systemic lupus erythematosus. 1125 92

This case report describes a patient with systemic lupus erythematosus who demonstrated by transesophageal echocardiography (TEE) a flail right coronary cusp of the aortic valve causing severe aortic regurgitation. This finding was confirmed at surgery, which showed near detachment of the right coronary leaflet near the commissure. Destruction of areas of the aortic valve has been reported in one previous case report in the absence of infective endocarditis or a traumatic process. This is the second case to our knowledge and the first to describe identification by TEE. (ECHOCARDIOGRAPHY, Volume 13, July 1996)
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PMID:Flail Aortic Valve in Systemic Lupus Erythematosus: Diagnosis by Transesophageal Echocardiography. 1144 51

Antiphospholipid antibodies are a heterogeneous family of immunoglobulins that includes lupus anticoagulant and anticardiolipin antibodies. They are strongly associated with a clinical syndrome characterized by venous and arterial thrombosis and spontaneous fetal losses. This syndrome may be primary or else secondary to autoimmune or neoplastic diseases. The cardiovascular system is frequently involved with mitral or aortic insufficiency, juvenile myocardial infarction, and primitive pulmonary hypertension. However, the occurrence of intracardiac thrombi is rare. We describe a case of an intracardiac right atrial thrombus in a 19-year-old asymptomatic woman who was admitted in December 1998 to the Thrombosis Center owing to the finding, during routine work-up, of a prolonged activated partial thromboplastin time (71 s) and thrombocytopenia (71 x 1000/mm3), a positive antinuclear antibody test (1/320), positivity for lupus anticoagulant, and increased IgG (92 GPL-U/ml) and IgM (27 MPL-U/ml) anticardiolipin antibodies. Six months later, the patient presented with headache, edema and cyanosis of the face and jugular swelling. Transthoracic and transesophageal echocardiography revealed a right atrial mass which was clearly distinguishable from the tricuspid valve and extended to the superior vena cava. The patient was successfully submitted to surgical excision of the thrombus. Histology revealed that the mass was adherent to an abnormal septum consisting of mesenchymal tissue. Although the American Rheumatology Association criteria for the diagnosis of systemic lupus erythematosus were not fulfilled, the positivity of antinuclear antibody test is in favor of a lupus-like syndrome. The decision to opt for surgical excision of the thrombus was determined by the unclear nature of the atrial mass. It may be necessary that such patients be submitted to anticoagulant therapy for the rest of their lives or temporarily (6-12 months). This underscores the importance of the anatomical abnormality as a promoting factor. Transthoracic echocardiography (as well as transesophageal echocardiography in selected cases) must be considered as an essential component of the initial diagnostic work-up in patients presenting with antiphospholipid antibodies.
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PMID:[Left atrial thrombosis in patients with antiphospholipid antibody syndrome and mesenchymal abnormal septum]. 1172 15

A 41-year-old woman diagnosed with aortoarteritis since 1988 was admitted with unstable angina. She also had anemia, thrombocytopenia, aortic regurgitation and pulmonary artery hypertension. She gave a history of recurrent fetal loss and myocardial infarction, following which angioplasty to the left anterior descending artery had been done. After investigation, a diagnosis of aortoarteritis with systemic lupus erythematosus and associated antiphospholipid antibody syndrome was made. Aortoarteritis may coexist with systemic lupus erythematosus and associated antiphospholipid antibody syndrome.
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PMID:Aortoarteritis with systemic lupus erythematosus and secondary antiphospholipid antibody syndrome: a rare association. 1221 30


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