Alternatively known as v2242ucous, marantic, or nonbacterial thrombotic endocarditis.
Cardiac manifestation of the autoimmune disease systemic lupus erythematosus.
Vegetations are reported autopsy studies of approximately 50% of fatal cases of lupus.
LSE attributed to 13-65% of premature SLE deaths at autopsy.
Associated with high level of disease activity and lupus nephritis.
Valvular vegetations reported in 7-11% of patients on transthoracic echocardiogram and 43% of patients on transesophageal echocardiography of unselected patients with SLE.
Also occurs in association with primary or secondary antiphospholipid syndrome, malignancy and hypercoagulable states.
Systemic lupus erythematosus and primary antiphospholipid syndrome occur 5-9 times more often in women.
Patients with cardiac valvular lesions are generally young women.
Left-sided valvular lesions are most common, and the mitral valve is more frequently affected than the aortic valve.
Autopsy findings of mulb2242ylike clusters of v2242ucae on the ventricular surface of the posterior mitral leaflet.
Pathologic findings often demonstrate adherence of the mitral leaflet and chordae to the mural endocardium with accumulations of immune complexes and mononuclear inflammatory cells.
Vegetations are fibrotic and or consist of proliferating endothelial cells mononuclear cell infiltrates or monocytes.
Valvular abnormalities occur as mitral leaflet thickening, valvular regurgitation, and, infrequently, stenosis.
The left-sided valves are involved most often with L-S endocarditis.
Transesophageal echocardiography of the mitral demonstrates abnormalities, although the proces is not always recognized on echocardiographic images.
Steroid therapy for systemic lupus erythematosus has improved longevity of patients and has changed the spectrum of valvular disease.
Process is usually asymptomatic, but may be manifested by valvular dysfunction, valve replacement, embolic phenomena, secondary infective endocarditis and heart failure.
Most commonly involves mitral and aortic valves, but all cardiac valves and the endocardial surfaces can be affected.
Valvular regurgitation is more common than stenosis.
Valvular stenosis is a rare manifestation of disease.
Echocardiographic studies reveal valvular abnormalities in 28-74% of patients.
Most vegetations are small and are not hemodynamically significant.
Valvular masses are identified by echocardiography in 4-43% of patients with systemic lupus erythematosus and valvular thickening is identified in 19-52% of cases.
Transesophageal echocardiography has a higher yield in diagnosisng abnormalities than does transthoracic echocradiography.
Mitral regurgitation is the most common valvular abnormality, followed by aortic regurgitation, combined mitral stenosis and regurgitation, and combined aortic stenosis and regurgitation.
Coexistent leaflet thickening, is present in 71% of patients with valve masses.
Valve abnormalities detected during echocardiography in patients with primary antiphospholipid syndrome has been reported in approximately 30% of cases, and is especially high in patients with peripheral arterial thrmoboses.
Risk of LSE increased by three among patients with SLE with antiphospholipid antibodies.
Heart failure develops in approximately 10% of patients who have valvular lesions.
Valve replacement is needed in 1-8% of patients.
Decreasing findings of this disorder at autopsy, and increased findings of healed lesions on pathological exam as a result of glucocorticoid therapy suggest therapeutic benefits of medical management of the disease.
Several longitudinal cohort studies, however, have found that immunosuppressive drugs are not related to valvular lesion changes and some study show worsening of lesions with glucocorticoid therapy.
Valvuloplasty with this process is associated with a high risk of complications.