EXTENDED FIBROTIC ENDOCARDITIS AS THE 1ST MANIFESTATION OF SYSTEMIC LUPUS-ERYTHEMATOSUS

Background. Cardiac abnormalities, such as myocarditis, pericarditis o r verrucous endocarditis (Libman-Sacks endocarditis) occur in about on e third of patients with systemic lupus erythematosus. This study desc ribes an unusual aspect of endocardial involvement. Case reports. Case no 1 : A 14 year-old girl was admitted 3 months after acute hemichore a because of heart failure plus biological inflammatory findings. Echo cardiography showed mitral insufficiency with enlargement of the left atrium and ventricle. There was some infiltration involving the endoca rdium of the left ventricle, the chordae tendinae and the mitral valve . The titres of anti-DNA and anti-nuclear antibodies were elevated whi le the serum hemolytic complement was depressed. Skin biopsy showed Ig G, IgM and Clq deposits along the dermoepidermal junction. Corticoster oids, diuretics and vasodilator drugs failed to completely cure the he art failure ; the mitral insufficiency required mitral valve replaceme nt 21 months later. Pathological examination showed extended fibrotic changes of the endocardium. Case no 2: A 4 year-old boy was admitted f or acute heart failure due to mitral insufficiency, associated with bi ological inflammatory findings. Echocardiography showed mitral insuffi ciency and enlarged left atrium and ventricle. Anti-DNA and anti-nucle ar antibody titres were elevated. The patient was given antibiotics fo llowed by corticosteroids and immunosuppressive drugs. The persistence of the mitral insufficiency required mitral valve replacement 7 month s later. Pathological examination showed fibrotic changes of the endoc ardium. Exacerbation of the inflammatory process was seen 2 months aft er surgery, with development of diffuse proliferative lupus nephritis. The patient died of kidney failure and neurological complications, 44 months after cardiac surgery. Conclusion. In both patients, the syste mic lupus erythematosus was revealed by endocardial involvement, a com plication that is usually seen later. The endocardium changes responsi ble for mitral insufficiency and requiring valve replacement in these two cases were different from those described as verrucous endocarditi s in classical forms of the disease.