[Hypertension and endocarditis in systemic lupus erythematosus--relationship between lupus nephritis and hypertension, Libman-Sacks disease].
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Mitral valve disease of systemic lupus erythematosus. A cause of severe congestive heart failure reversed by valve replacement.
Libman-Sacks endocarditis caused progressive life-threatening mitral regurgitation necessitating mitral valve replacement in an 18 year old woman with systemic lupus erythematosus (SLE). Although Libman-Sacks endocarditis is frequently seen at autopsy in patients with SLE, hemodynamically significant valvular disease due to that lesion is quite rare. We found no previous reports describing mitral regurgitation in a patient with SLE which has necessitated surgical intervention.
Adolescent↗
Stroke in systemic lupus erythematosus.
We investigated the clinical and pathologic characteristics of stroke in 234 patients with systemic lupus erythematosus. Thirteen patients (5.6%) developed cerebrovascular disease. Cerebral infarction was noted in eight, cerebral hemorrhage in two, and subarachnoid hemorrhage in three. In seven (54%) of these 13 patients, stroke occurred less than or equal to 5 years after systemic lupus erythematosus was diagnosed. Among the predisposing risk factors for stroke, hypertension was the most important. Lupus anticoagulant was detected in three (38%) and anticardiolipin antibody in three (43% of seven investigated) of the patients with infarction. Evaluation of the clinical manifestations and autoantibodies indicated that renal involvement and high titers of anti-deoxyribonucleic acid antibody were more frequent in the stroke group than in the non-stroke group. Autopsy studies on six of the patients with stroke revealed small infarcts and hemorrhages in all, but in no case was true angiitis observed. Libman-Sacks endocarditis was found in two of the three patients with infarction. In conclusion, the important contributory factor to the development of stroke in patients with systemic lupus erythematosus is considered to be hypertension mediated by immunologic abnormalities. Antiphospholipid antibodies and Libman-Sacks endocarditis are closely associated with occlusive cerebrovascular disease.
Adolescent↗
Clinically significant valvular heart disease in systemic lupus erythematosus.
PURPOSE: Clinically significant valvular heart disease due to systemic lupus erythematosus (SLE) has generally been considered rare, and Libman-Sacks endocarditis has been thought to be predominantly an autopsy finding. With the declining prevalence of rheumatic heart disease, however, the spectrum of valvular heart disease is changing. We retrospectively analyzed our experience with SLE between 1975 and 1987 for the presence of hemodynamically significant valvular heart disease. PATIENTS AND METHODS: An existing data base of 421 patients with SLE was selected for review. Patients were selected for inclusion in the study if they met four or more of the criteria of the American Rheumatism Association for SLE, they had clinically significant valvular heart disease, and tissue from the involved valve was available for review. The etiology of the valve lesion was determined by assessment of the clinical history, chart review, gross morphology, and valve histology. RESULTS: Of 14 cases with pathologic material available for review, six had anatomic features of SLE valvular heart disease such as verrucous vegetations or valvulitis with necrosis and vasculitis. Two of these patients underwent successful valve replacements and four died from complications of their valve disease. CONCLUSION: We suggest that significant morbidity and mortality may result from SLE valvular heart disease in about 1 to 2 percent of SLE patients and that the pathogenetic mechanisms underlying valve dysfunction in SLE patients are multifactorial.
Adolescent↗
Clinical and neuropathological findings in systemic lupus erythematosus: the role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura.
We reviewed the medical records and autopsy reports of 50 patients with systemic lupus erythematosus to determine the clinical and neuropathological features of this disease. Neuropsychiatric disturbances were found in the majority (74%) of the patients, occurring as psychiatric illness only (5 patients), neurological disorders only (15 patients), and both together (17 patients). Central nervous system (CNS) lesions were present in half the patients; embolic brain infarcts (10 patients) and CNS infections (8 patients) were the most common. Cardiac sources of emboli were Libman-Sacks endocarditis (5 patients), chronic valvulitis (2 patients), and left side of heart mural thrombus (2 patients). There was no evidence of active CNS vasculitis. Clinical features of thrombotic thrombocytopenic purpura (TTP) developed during the terminal illness in 14 patients, 7 of whom also had pathological evidence of TTP. Correlation between neuropsychiatric disorders and brain lesions could be made in approximately half the patients. This study indicates that cardiac emboli from Libman-Sacks endocarditis and TTP are common pathogenetic factors of CNS disease in systemic lupus erythematosus, whereas CNS vasculitis is rare.
Adolescent↗
Cardiac abnormalities in systemic lupus erythematosus: prevalence and relationship to disease activity.
We conducted a prospective longitudinal study to determine the nature and prevalence of cardiac abnormalities in systemic lupus erythematosus and to study their natural history and relationship with disease activity. Forty consecutive inpatients with systemic lupus erythematosus were studied during their admission and subsequently 6 to 12 months later. On each occasion a clinical cardiovascular examination was carried out, disease activity was scored using the "Lupus Activity Criteria Count" and a Doppler echocardiographic examination was carried out. 72.5% of patients had an abnormal echocardiogram in the first study while 51.7% were abnormal during the follow-up study. Valvar disease occurred in 37.5% of patients. The mitral valve was most commonly affected. Libman-Sacks endocarditis was rare (2.5%). Pericardial effusions were seen in 36.2% of echocardiograms. The majority (76.0%) of these were associated with hypoalbuminaemia. 80.0% of patients had active disease during the first examination and 41.4% at follow-up. There was no correlation between activity of disease and prevalence of cardiac abnormalities at either examination. We conclude that cardiac disease is common in systemic lupus erythematosus. Prevalence of cardiac abnormality did not correlate with disease activity.
Adolescent↗
Double valve disease in systemic lupus erythematosus. Case report and literature review.
A young, nonhypertensive female with advanced systemic lupus erythematosus (SLE) presented in congestive cardiac failure due to aortic and mitral regurgitation. The valvular lesions resulted from organization of valvular pocket Libman-Sacks vegetations. Her clinical course mimicked infective endocarditis. She is only the third recorded patient with SLE valvular disease warranting double valve replacement. This patient, who had her valvular disease at presentation (prior to initiation of steroid therapy), illustrates that untreated SLE per se may produce severe organic valvular disease.
Adolescent↗
Mitral stenosis in systemic lupus erythematosus. Successful management by mitral valve replacement.
A female Caucasian with a history of 18 years of systemic lupus erythematosus (SLE) developed symptoms and signs of mitral stenosis, but had no history of rheumatic fever. Investigations confirmed severe stenosis, and the diseased valve was replaced by a pericardial xenograft. Histological examination was compatible with steroid-modified Libman-Sacks endocarditis. She remains well 24 months postoperatively.
Adult↗
[Evolution and current characteristics of the clinical picture and course of systemic lupus erythematosus in children].
Altogether 506 patients with systemic lupus erythematosus (SLE) aged one to fifteen years were observed from 1961 to 1985 at the clinic of child's diseases of the I. M. Sechenov First Moscow Medical Institute. The disease was noted to undergo evolution during 25 years. The clinical picture and the course of SLE in children were elucidated. It is shown that the disease activity and acuity lessened while the patients' survival increased. The clinical picture became less marked. The rate of the signs characteristic of SLE (Libman-Sacks endocarditis, the nephrotic syndrome, severe lesions of the nervous system, exudative pleurisy and pericarditis) also reduced. The characteristics thus revealed should be taken into consideration in the diagnosis and treatment of SLE.
Acute Disease↗
Association of lupus anticoagulant with severe valvular heart disease in systemic lupus erythematosus.
Two cases of systemic lupus erythematosus with hemodynamically significant mitral valve dysfunction and associated lupus anticoagulant are reported. Both patients underwent valve replacement and both had thrombus formation on the mitral valve, one pre- and the other postoperatively. Both patients suffered a number of extracardiac thromboses at different times in the course of their illness. The contribution of the lupus anticoagulant to the thrombotic problems, and its possible relationship to the pathogenesis of Libman-Sacks endocarditis are discussed.
Adolescent↗
[Heart valve involvement in systemic lupus erythematosus: an echocardiographic study].
The purpose of this study was to evaluate the spectrum of morphologic and functional cardiac involvement in a selected population of patients with systemic lupus erythematosus (SLE) by means of echocardiography. Thirteen patients (2 male and 11 female) affected by SLE, mean age 41.9 years (range, 21-64), underwent M-Mode, two-dimensional and Doppler echocardiography. Eleven patients had renal disease and 3 of them were undergoing dialysis. One patient had findings of active disease. Six patients had systemic hypertension. None had a history suggestive of rheumatic fever or infective endocarditis. At echocardiographic study nine patients demonstrated findings of valvular involvement. These alterations were defined, according to the echocardiographic features, in two types: vegetation (verrucous Libman-Sacks endocarditis) and thickening. Vegetations were present in 6 patients, involving the mitral valve in all six and the aortic valve in three. The mitral valve vegetations were more frequent on the subannular portion of the posterior leaflet. Seven patients had valvular thickening: involvement of both mitral and aortic valve was present in five, and isolated mitral or aortic valve lesions in the remaining two patients. Combined valvular vegetation and thickening were observed in 4 patients. Eight patients had mild valvular dysfunction on Doppler examination: five isolated mitral regurgitation, two combined mitral and aortic regurgitation and one combined mitral stenosis and regurgitation. In agreement with previous reports, our study shows that valvular involvement in SLE is relatively frequent. Echocardiography can identify additional patterns of valvular lesions different from the known "verrucous Libman-Sacks endocarditis". The degree of valvular dysfunction is not important.
Adult↗
Systemic lupus erythematosus as a cause of severe mitral regurgitation. New problem in an old disease.
Clinical and morphologic observations in three patients with systemic lupus erythematosus and severe mitral regurgitation are described. Attention is called to the "healing" of Libman-Sacks endocarditis, an infrequent occurrence in patients with systemic lupus erythematosus in the era before steroid therapy. The mitral regurgitation in our patients appears to have resulted from "healing" of the Libman-Sacks vegetations by scarring and calcification. The healing is attributed to long-term corticosteroid therapy.
Adolescent↗
[Echocardiographic analysis of changes in the heart in patients with systemic lupus erythematosus].
Fifty-six patients, 49 females and 7 males, with the confirmed diagnosis of systemic lupus erythematosus were examined by M-mode, 2--D and Doppler echocardiography. Pericardial effusion was found in 15 patients (27%), while pericardial thickening was suspected in 6 additional patients (37.5% altogether). Two patients had the signs of a pericardial tamponade, but both of them were uraemic. Libman-Sacks endocarditis was suspected in 4 patients (7.5%) because of verrucous changes in the aortic or mitral valve and regurgitant jet. Slight to moderate left ventricular hypocontractility was present in 3 patients (5%), while 3 additional patients had borderline values of the left ventricular contractility parameters. Left ventricular hypertrophy, usually mild, was found in 21 patients (37.5%). Echocardiographic signs of pulmonary hypertension were present in 2 patients (3.6%). It has been concluded that pericardial affection is frequent during the course of systemic lupus erystematosus, while a diffuse myocardial involvement is rare, except the consequences of arterial hypertension and accelerated coronary atherosclerosis. Libman-Sacks endocarditis still represents a diagnostic problem. For a more precise definition of cardiac involvement in systemic lupus erythematosus, a comparative analysis of the disease activity and immunosuppressive therapy is needed.
Adolescent↗
Cardiovascular involvement in systemic lupus erythematosus.
SLE is an inflammatory disease of unknown etiology with the potential of affecting virtually all organ systems. Cardiovascular involvement occurs frequently, although it is often mild enough not to cause clinical concern. Pericarditis is most commonly subclinical, noted only on echocardiogram. Pericardial fluid, which can accumulate rapidly enough to cause tamponade, is inflammatory in nature and can totally mimic infection. The occurrence of Libman-Sacks endocarditis, usually a pathological diagnosis of little clinical significance, has little if any correlation with the presence of audible murmurs. However, valve replacement is occasionally necessary secondary to sterile destruction. These valvular lesions can also embolize or become infected. The incidence of ischemic coronary disease is increased, both secondary to premature atherosclerosis and, rarely, coronary arteritis. Conduction disease and arrhythmias are infrequently reported in adult patients, but congenital CHB has been noted in children born to mothers who have circulating anti-Ro antibody. Evidence is accumulating that suggests there is a mild cardiomyopathy associated with SLE that may be due to thrombotic or inflammatory microvascular coronary disease. Acute clinical myocarditis also rarely occurs. Therapeutically, at present, a reasonable course would seem to be to limit all known possible contributing factors to premature coronary artery and myocardial disease (hypertension, hypercholesterolemia, smoking, steroid therapy, etc), to be vigilant about recognizing the rarer complications associated with SLE (infectious pericarditis and endocarditis, coronary arteritis, pericardial tamponade, clinical myocarditis), and to remember that these uncommon complications are indeed uncommon. The importance of vigorously treating systemic hypertension cannot be overstressed.
Cardiovascular Diseases↗
Pancreatitis related to antiphospholipid antibody syndrome in a patient with systemic lupus erythematosus.
A 39-year-old woman was admitted with abdominal pain and dyspnea, and a diagnosis of systemic lupus erythematosus with renal involvement was established. Laboratory tests revealed highly elevated anticardiolipin antibody, thrombocytopenia and false positive VDRL. Generalized thrombus formation and Libman-Sacks endocarditis were found at postmortem examination. The pancreas showed chronic inflammation with thrombi in pancreatic arteries, but no vasculitic change was observed. Lowering of pancreatic blood flow because of arterial thrombi was a possible cause of pancreatitis in this patient. The spectrum of antiphospholipid antibody associated diseases may be extended to include pancreatitis as a thrombotic complication.
Adult↗
Aortic insufficiency and mitral regurgitation in patients with systemic lupus erythematosus and the antiphospholipid syndrome.
PURPOSE, PATIENTS, AND METHODS: Heart disease has not been well characterized in patients with systemic lupus erythematosus (SLE) and the antiphospholipid syndrome. During a prospective study of cerebrovascular disease in autoimmune disease and SLE, 11 lupus patients were identified with an antiphospholipid syndrome characterized by significant cardiac valvular disease in addition to cerebral infarction, deep vein thromboses, and thrombocytopenia. Patients were reviewed for criteria for systemic lupus and underwent echocardiographic studies and measurements of anticardiolipin antibodies, VDRL, and the lupus anticoagulant. RESULTS: Eight of the 11 patients had aortic insufficiency, two of whom had associated mitral regurgitation. Three patients had mitral regurgitation alone. Microscopic analysis of a surgically excised aortic valve indicated typical Libman-Sacks verrucous endocarditis. Infective endocarditis was ruled out in all patients. CONCLUSION: This report expands previous descriptions of antiphospholipid syndromes by describing a subset of lupus patients with significant aortic and mitral valvulitis in addition to circulating antiphospholipid antibodies, thrombocytopenia, and recurrent thromboses.
Adult↗
Bioprosthetic valve excision without replacement in the tricuspid position in a patient with Libman-Sacks endocarditis.
A 19 year old girl with Systemic Lupus Erythematosus (SLE), in chronic renal failure and on haemodialysis, underwent tricuspid valve replacement with a Carpentier-Edwards xenograft because of a large calcified mass invading the right side of the heart. The mass caused no symptoms and had been discovered accidentally on a routine chest X-ray. The bioprosthetic valve had subsequently to be removed, having developed functional stenosis, and was not replaced. Despite the absence of a tricuspid valve, the patient remains well five years after the operation.
Adult↗
Echocardiographic diagnosis of bland thrombus on an aortic valve.
Echocardiography is a valuable modality for imaging thrombi and vegetations. Although patients with systemic lupus erythematosus may develop Libman-Sacks endocarditis, in which punctate verrucous plaques are found histologically on valvular endocardial tissue, these lesions are ordinarily too small for ultrasonic imaging. We describe a patient with systemic lupus erythematosus complicated by systemic embolization, who had a large mobile aortic valve thrombus, which was discovered with the aid of echocardiography and was confirmed by surgical resection.
Adult↗