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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This case involves a 41-year-old woman with SLE. The patient began having symptoms of arthralgia in 1978 and developed fever, pleuritis and lupus psychosis in 1986. Laboratory exams showed positive antinuclear-antibody, LE-cell phenomenon, hypocomplementemia and lupus anticoagulant. Echo cardiography demonstrated mitral regurgitation and stenosis. She was treated with 50 mg of prednisolone and these manifestations subsided. In 1989, she developed dyspnea on exertion and echo cardiography revealed severe mitral stenosis. Pulmonary infarction was detected by MAA lung scintigraphy. At this time, she was diagnosed as SLE associated with antiphospholipid syndrome (APS). A mitral valvular replacement operation was performed in 1991. Pathological studies of mitral valve demonstrated Libman Sacks endocarditis. APS is known occasionally to complicate with left-sided valvular diseases, mitral stenosis is quite rare in both SLE and APS. This patient reveals a rare case of SLE associated with APS and mitral stenosis. It is suggested that this patient developed mitral stenosis with Libman Sacks endocarditis, associated with the presence of antibody against phospholipids.
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PMID:[A case of SLE associated with antiphospholipid syndrome and mitral stenosis]. 755 44

Valvular replacement was performed in a patient with primary antiphospholipid syndrome and severe mitral insufficiency. The valvular tissue presented thrombotic phenomena and fibrosis with minimum inflammatory component.
Lupus 1994 Oct
PMID:Severe valvular heart disease in a patient with primary antiphospholipid syndrome. 784

Valvulitis in systemic lupus erythematosus has been observed for many years. Fourteen cases of mitral valve replacement have been reported so far in the literature. In the light of all previously reported experiences, prosthetic valve related morbidity and mortality remain high in patients with systemic lupus erythematosus. We describe the first true instance of reconstructive approach in a patient who presented with severe mitral incompetence due to lupus valvulitis. The patient was clinically well with good valve function one year after surgery. Reconstructive mitral valve surgery may be preferable for the surgical management of mitral regurgitation in active lupus valvulitis since it eliminates the need for anticoagulation and avoids the disadvantages of different types of prostheses in these young patients who are under prolonged steroid therapy and have usually associated renal failure.
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PMID:Mitral valve repair in lupus valvulitis--report of a case and review of the literature. 855 85

For many years, valvulitis in systemic lupus erythematosus has been known to occur. Our patient was a 17-year-old girl who presented with severe mitral incompetence and renal insufficiency due to lupus valvulitis. The patient was first treated by mitral valve repair, but follow-up disclosed precocious calcification of the valve, necessitating mitral valve replacement with a cryopreserved homograft. At follow-up after 1 year, echocardiography has shown the valve to be functioning normally. A reconstructive mitral valve operation would seem to be preferable. However, a conservative operation does not alter the natural history of the disease and the progression of valve thickening and calcification.
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PMID:Systemic lupus erythematosus valvulitis: mitral valve replacement with a homograft. 878 90

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

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon syndrome belong to close but different subsets of Sneddon syndrome.
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PMID:Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients. 1042 3

Many patients with systemic lupus erythematosus (SLE) develop cardiac manifestations during the course of their disease. Pericarditis is most commonly seen, with a reported prevalence of 60%. Myocardial involvement is present in only a minority of patients. In recent years, due to better noninvasive diagnostic techniques, valvular abnormalities can be demonstrated in an increasing number of patients. Depending on the technique used, valvulopathy can be demonstrated in up to 77% of SLE patients. Although most of the valvular lesions will be present without any symptoms, valve incompetence can result in congestive heart failure. Valvular lesions are associated with IgG anticardiolipin antibodies (aCL) and disease duration. We present a patient with SLE and secondary antiphospholipid syndrome (APS) who developed acute congestive heart failure due to pancarditis. Endocarditis, together with left ventricular dysfunction and pericardial effusion, were present. The endocarditis caused hemodynamically significant mitral valve insufficiency due to thickening of the mitral cusps. Just two weeks prior to the occurrence of congestive heart failure echocardiography had been normal. Treatment with high dose corticosteroids resulted in a gradual, almost complete recovery. Literature concerning cardiac manifestations in lupus is reviewed.
Lupus 2000
PMID:Cardiac abnormalities in SLE: pancarditis. 1086 93

We report an illustrative case of a 60-year-old man with Streptococcus viridans subacute bacterial endocarditis (SBE) and positive antineutrophil cytoplasmic autoantibodies (c-ANCA). C-ANCA positivity has been associated with a variety of rheumatic and infectious disease areas, but has been rarely associated with SBE. The patient had mitral valve prolapse with mitral regurgitation, and S viridans SBE developed after a dental procedure. Laboratory abnormalities included anemia, elevated erythrocyte sedimentation rate, positive rheumatoid factor, positive anticardiolipin antibody, positive lupus anticoagulant, and highly elevated c-ANCA level. We believe this is only the ninth reported case of S viridans SBE with a positive c-ANCA, and the third with mitral valve prolapse and vegetations.
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PMID:Streptococcal viridans subacute bacterial endocarditis associated with antineutrophil cytoplasmic autoantibodies (ANCA). 1273 37

Systemic lupus erythematosis (SLE) is an autoimmune disease frequently accompanied by the presence of an antiphospholipid antibody (APA). Early referred to as the lupus anticoagulant (LAC), this APA consists of immunoglobulins that are known to interfere with coagulation tests that are phospholipid dependent. Such tests include the partial thromboplastin time (PTT), the activated clotting time (ACT) and may affect the thrombin time (TT). This challenges the cardiac surgical team and the perfusionist responsible for monitoring anticoagulation while performing cardiopulmonary bypass (CPB). A 46-year-old female with a history of SLE, severe mitral insufficiency, an anterior wall myocardial infarction, and the presence of a LAC was admitted for mitral valve surgery. Replacement of the mitral valve was accomplished successfully, utilizing CPB. Anticoagulation was managed using the Hepcon HMS PLUS, a device that calculates an individual's heparin dose response and permits assessment of the heparin concentration throughout the procedure. The patient recovered and was sent home 16 days after surgery.
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PMID:A case report of mitral valve replacement in a patient with lupus antibody syndrome. 1471 75

Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order to compare demographic features and cardiological outcome of patients with ICHB submitted to pacemaker (PM) implantation with and without NLS, forty ICHB patients were evaluated pre- and post-PM implantation, by clinical, electrocardiogram, Holter Monitoring, treadmill test, and electrophysiological study. According to the presence of antibodies to 52 and 60 kDa Ro/SSA and La/SSB proteins in mother's sera, it was found that 60% (24/40) of patients had ICHB associated to NLS (ICHB/NL+). Twenty-three of 24 ICHB/NL+ patients were asymptomatic, and 16 (67%) were female (P = 0.013). The frequency of syncope, mitral insufficiency (MI), and congestive heart failure (CHF) was similar pre-PM implantation in both ICHB/NL+ and ICHB/NL- groups (P > 0.05). After PM implantation, MI and CHF were only observed in ICHB/NL+ patients, although not statistically significant. Interestingly, 67% of ICHB/NL+ were noticed before one year of age while only one fourth of ICHB/NL- was diagnosed in this period (P = 0.024). Almost half (46%) of ICHB/NL+ patients required PMs in the first 24 months of life, whereas only one in the ICHB/NL- received a PM at the same age (P = 0.02). In ICHB patients requiring PM implantation, the antibody-mediated lesion seems to be associated with an earlier onset and a more severe heart disease, in spite of the uniform criteria for PM indication.
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PMID:Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome? 1512 17


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