Libman-Sacks endocarditis (LSE) is a rare disease found incidentally in postmortem autopsies, characterized by microscopic to large verrucous vegetation on the cardiac valves, the most affected site is the mitral valve followed by the aortic valve. Females of reproductive age were observed as the most affected individuals as found in studies. Most individuals with LSE are asymptomatic and generally discovered lately when they presented with thromboembolic disorders such as stroke, cognitive disabilities, and death. Malignancy and autoimmune diseases involving systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are considered the primary etiology of LSE. As recognized, the majority of LSE cases are asymptomatic, it tends to be challenging to spot the condition at the early pathway of the disease. In this paper, we describe a young female who is known to have SLE on medications, she presented to the emergency department (ED) due to chest pain and exertional dyspnea for a few days, laboratory investigations showed anemia, raised inflammatory marker, and anti-DsDNA. Imaging studies showed bilateral pleural effusion on the chest X-ray and a large vegetation on the posterior mitral valve with moderate regurgitation and normal wall motion in transesophageal echocardiography. The patient was managed by pulse steroid therapy, anticoagulation therapy, and a low dose of diuretic, the patient improved dramatically and discharged home with close follow-up in the clinic. The primary treatment of LSE is anticoagulant therapy, however, surgical intervention should be considered in case of large vegetation recurrent thromboembolism despite anticoagulant therapy. As the prognosis in LSE is considered very poor and there is no definitive laboratory investigation exists to confirm the diagnosis, we highlight the importance of considering LSE as a serious and crucial differential diagnosis when dealing with SLE patients who presented with dyspnea and pleural effusion secondary to valvular dysfunction, mainly the mitral valve.
Keywords: anticoagulant therapy; libman sack endocarditis; mitral valve vegetation; systemic lupus erythematous; warfarin.
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