Revisiting Libman–Sacks Endocarditis: A Historical Review and Update

Abstract

Libman-Sacks (LS) endocarditis was first described by Libman and Sacks in 1924, and is characterized by sterile, verrucous valvular lesions with a predisposition for the mitral and aortic valves. It is now regarded as both a cardiac manifestation of systemic lupus erythematosus and, in recent years, of the antiphospholipid syndrome (APS). Though typically mild and asymptomatic, LS endocarditis can lead to significant complications, including severe valvular insufficiency requiring surgery, infective endocarditis, and thromboembolic events, such as stroke and transient ischemic events. Improvements in imaging modalities, particularly in echocardiography, have allowed better estimation of the prevalence of the disease, but further investigation is still needed into its pathogenesis, treatment, and association with APS.

Endocardite de Libman-Sacks, anticorpos antifosfolípides e trombose arterial no lúpus ertitematoso sistêmico: relato de caso

“…Very high CRP levels suggest an infectious cause, as lupus patients are less capable of presenting an exuberant response of this protein; however, for a definitive differential diagnosis, the blood cultures are more important. 2 In the present case, a diagnosis of LSE was attained, as the leukocyte count was normal, the CRP was not very elevated, APA was positive and six pairs of blood culture samples had negative results.…”

“…In this aspect, three laboratory data are important: leukocyte count, CRP levels, and APA levels. 2 The leukocytes tend to decrease during lupus activity and the opposite occurs in infectious endocarditis. As for the APAs, it is unlikely that elevated levels be caused by infectious diseases, thus suggesting the presence of SLE.…”

“…1,2 Patients with moderate to high titers of anticardiolipin IgG antibodies have a higher incidence of valve lesions, whereas there are patients with valve disease in whom lupus anticoagulant are the only anti-phospholipid antibodies detected. 2 More recently, moderate to high titers of anti-b2-glycoproten-I antibodies have been associated with valve disease in APS 7 . In our service, we do not perform the screening for this antibody, but we emphasize the importance of its assessment in patients suspected to have APS.…”

“…1 It is usually asymptomatic; however, cases that needed valve replacement have been described. 2 An association between LSE and antiphospholipid antibodies (APAs), with an increased risk of thromboembolic phenomena, has been observed in these patients. 1 The present case report describes a patient with SLE, LSE, and antiphospholipid syndrome (APS) that had an acute peripheral arterial thromboembolic event.…”

Accidente cerebrovascular embólico por endocarditis de Libman-Sacks como manifestación inicial de síndrome antifosfolípidos primario en una paciente de 17 años: Report of one case

“…Por la naturaleza autoinmune del SAF, es esperable que existan fenómenos inflamatorios implicados en la patogenia de las manifestaciones clínicas. Se postula que en pacientes con ELS (con LES y/o SAF), los aCL estimularían la formación del trombo sobre un endotelio valvular donde se han depositado previamente complejos inmunes, exacerbando un daño inflamatorio en la válvula 8 . En un consenso internacional de expertos del año 2005 para la actualización de criterios diagnósticos del SAF 1 , se otorga una definición de lo que se considera compromiso valvular y cardiaco por SAF, pero se aboga en contra de considerarlo como un criterio adicional por la existencia de factores confundentes relacionados con enfermedades cardiovasculares en gran parte de los pacientes.…”