Case Presentation: A 26-year-old woman with systemic lupus erythematosus (SLE) of ten years (status post renal transplant complicated by secondary acute rejection) presented to the Emergency Department with a week and a half of bilateral lower extremity edema and nausea. The patient’s lower extremity edema began nine days prior to admission and was slowly worsening. Upon evaluation, she endorsed fatigue and dyspnea on exertion but denied orthopnea, shortness of breath, and chest pain. Vital signs on admission were unremarkable (saturating >95% on room air). Relevant physical exam findings included 2+ bilateral symmetric edema. Labs were remarkable for acute on chronic anemia (hemoglobin 6.8), elevated proBNP (12,799), and low calcium (6.2); there were no other electrolyte abnormalities. She endorsed adherence to nightly peritoneal dialysis sessions and her medication regimen, which included Methotrexate 400 mg three times per week and Prednisone 5 mg daily.Initial differential of acute-onset lower extremity edema included new-onset heart failure vs. renal disease with insufficient dialysis vs. peripheral valvular insufficiency. In consult with the nephrology team, the patient received nightly peritoneal dialysis sessions while inpatient. Subsequent work-up, including a trans-thoracic echocardiogram (TTE), revealed left ventricular ejection fraction (LVEF) of 52.7% with moderate-severe mitral regurgitation (MR) and thickening of the mitral valve leaflet tips. The patient was evaluated for bacterial endocarditis; however, she lacked fever and vascular/immunologic phenomena, and blood and peritoneal fluid cultures were negative. Given her history of systemic lupus erythematosus, acute-onset MR, and lack of symptoms concerning for bacterial endocarditis, presentation was consistent with Libman-Sacks endocarditis (LSE).Although patient met criteria for mitral valve repair/replacement, she was ultimately treated with oral anticoagulation after extensive discussions between the patient and rheumatology and cardiology teams (1). Risks and benefits of medical treatment were discussed, and surgical repair remained an option if symptoms persisted or worsened. No changes were made to her SLE regimen given lack of active symptoms. The patient’s lower extremity edema and nausea slowly improved during admission. She was discharged five days after admission and referred to outpatient cardiology.
Discussion: Cardiac manifestations of systemic lupus erythematosus (SLE) are common, with many patients developing complications such as pericarditis or cardiac lesions (2). Patients with SLE are at increased risk of developing Libman-Sacks endocarditis (LSE), a form of nonbacterial thrombotic endocarditis (3). Although patients may be asymptomatic, LSE most commonly involves the mitral and aortic valves and may lead to valvular insufficiency and thromboembolic events. Treatment of underlying rheumatologic disease and anti-coagulation is strongly recommended, especially in patients with lupus and/or positive lupus anticoagulant (5). Patients who poorly respond to medical therapy or with severe valvular abnormalities should undergo surgical evaluation (6).
Conclusions: Identifying Libman-Sacks endocarditis can pose a unique diagnostic challenge. Understanding LSE risk factors and clinical presentation can facilitate early recognition, leading to treatment of the disease and incorporation of preventative measures to reduce morbidity.