Cox’s Conference: Libman-Sacks Endocarditis
In this week’s Ambulatory Cox’s Conference, Dr. Vishnu Prathap presented a case of a female with history of systemic lupus erythematosus (SLE) who presented with a chief complaint of shortness of breath. Our expert discussant and program director, Dr. Dino Kazi guided us through the differential diagnosis for shortness of breath in a patient with SLE.
In a patient with SLE, the differential for shortness of breath is broad due to the wide range of clinical manifestations. Our differential diagnosis included thromboembolic disease (due to possible antiphospholipid syndrome), renal failure (possible lupus nephritis), pulmonary vasculitis, pleuritis with effusion, interstitial lung disease, pulmonary hypertension, myocarditis/pericarditis, pericardial effusion, premature coronary artery disease, and valvular heart disease. Our patient was found to have vegetations on both the posterior and anterior leaflets of the mitral valve with associated severe mitral stenosis. She was diagnosed with Libman-Sacks Endocarditis and the shortness of breath was attributed to valvular heart disease.
Libman-Sacks Endocarditis Pearls:
- Sterile, valvular vegetations of fibrous tissue made up of proliferating endothelial cells, myocytes, and mono-nuclear cells.1
- Valvular vegetations are not uncommon in patients with SLE. In a study of asymptomatic patients with SLE, valvular vegetations were found in 7 to 11% of patients on transthoracic echocardiography and 43% of patients on transesophageal echocardiography .2,3
- Left sided valves are most commonly affected with the mitral valve more commonly involved.
- Risk factors include presence of lupus nephritis, antiphospholipid antibodies, and high disease activity.
- Often times the vegetations are small and clinically silent, however some patients can develop significant valvular disease, valvular heart failure, or systemic embolic phenomena.
- Severe valvular regurgitation and heart failure symptoms occur in ~ 10% of patients with SLE who have valvular lesions.3,4
- Thorough history and physical exam (cardiac murmurs, splinter hemorrhages, or other evidence of embolization)
- Echocardiography – transesophageal echocardiogram more sensitive
- Rule out infectious endocarditis with blood cultures
- Anticoagulation is indicated if there is no contraindication and should be continued indefinitely.
- Surgical intervention in select cases.
- Treatment of underlying disease. However steroids or cytotoxic therapy have no known effect on the valvular lesions.
- Antibiotic prophylaxis is appropriate during dental procedures or when patients are suspected to be transiently bacteremic.
- Eiken PW, Edwards WD, Tazelaar HD, McBane RD, Zehr KJ. Surgical pathology of nonbacterial thrombotic endocarditis in 30 patients, 1985-2000. Mayo Clin Proc 2001;76:1204-1212
- Moyssakis I, Tektonidou MG, Vasilliou VA, et al. Libman-Sacks endocarditis in systemic lupus erythematosus: prevalence, associations, and evolution. Am J Med 2007;120:636-642
- Roldan CA, Shively BK, Crawford MH. An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus. N Engl J Med 1996;335:1424-1430
- Tarter L, Yazdany J, Moyers B, et al. The Heart of the Matter. N Engl J Med 2013; 368:944-950.