Multiple embolic strokes as a result of Libman-Sacks endocarditis associated with lupus and secondary antiphospholipid antibody syndrome: a case report

Abstract

Background: Libman-Sacks endocarditis (LSE) is an infrequently recognized pathogenesis of embolic cerebrovascular disease. Patients often have asymptomatic valvular dysfunction which if not recognized promptly, can lead to serious complications such as heart failure, arrhythmias, cerebroembolic phenomena with increased neurocognitive disability, and even death. It can be associated with systemic lupus erythematosus and/or antiphospholipid antibody syndrome (APLS).

Case summary: Previously very healthy and active, 49-year-old Caucasian female with past medical history of mild lupus, for which she stopped treatment 10 year ago, saw a primary care physician complaining of intermittent double vision of 2 months duration. Urgent brain magnetic resonance imaging revealed multiple embolic infarcts of the brain stem. Further comprehensive work-up led to diagnosis of mitral LSE and APLS. After 2 months of systemic anticoagulation with warfarin and immunosuppressive therapy with hydroxychloroquine sulfate, repeat imaging demonstrated resolution of the mitral valve vegetation with no clinical recurrence of thromboembolic events at 6 months.

Discussion: Mild, often silent, autoimmune disease as described in our case can lead to significant cerebrovascular disease. Patients who present with cryptogenic strokes with high suspicion of underlying autoimmune disease should be worked up thoroughly for possible valvular heart disease associated with lupus, APLS, or both. Acquisition of transoesophageal images proved superior to transthoracic approach and it should be implemented in these subsets of patients. With this case report, we highlight the importance of early recognition of cardiac manifestations, amelioration of risk factors, as well as close follow-up of lupus or APLS patients, as crucial steps in reducing their morbidity and mortality along with preventing recurrence or progression of cerebrovascular disease.

Keywords: Antiphospholipid syndrome; Case report; Non-bacterial endocarditis; Systemic lupus erythematosus.

Figure 1

Initial transoesophageal two-chamber view demonstrating several small sessile, loosely organized, fixed mitral vegetations with moderate regurgitation directed eccentrically.

Figure 2

Follow-up transoesophageal two-chamber view shows resolution of previous mitral valve vegetation and regurgitation.

Figure 3

(A) Mitral valve infectious vegetation most commonly located on atrial coaptation side. (B) Cardiac masses such as papillary fibroelastoma may mimic vegetation, but they are typically located on the left ventricular side of mitral valve. (C) Non-infectious mitral valve vegetations can be variable in shape and size typically smaller than infectious and can affect any part of the leaflets, but with tendency to occur more often near the leaflet base.