Background/Purpose:        

Libman-Sacks endocarditis (LSE) is a rare manifestation of pediatric-onset systemic lupus erythematosus (pSLE). It is difficult to differentiate LSE from infective endocarditis (IE) in immunosuppressed patients. Data remain scarce on the diagnosis of LSE and disease course. We aim to describe the diagnosis, evaluation and management of endocarditis in pSLE.

Methods:

A retrospective chart review was conducted of all patients who fulfilled ACR lupus classification criteria and were diagnosed with pSLE and either LSE or IE. Data were collected on demographics, laboratory results, echocardiogram findings, clinical features, and treatment. Descriptive statistics were used.

Results:

We describe 6 patients (4 females) with pSLE and endocarditis, with a mean age at diagnosis of 11.6 +/- 3.3 years (Table 1). Three patients were White, 2 Black, and 1 Asian. The mean disease duration from pSLE diagnosis to diagnosed cardiac involvement was 1.4 +/- 2.8 years. Four patients had biopsy-proven lupus nephritis. All patients had positive serologies for ANA and anti-dsDNA antibodies. Five patients had anti-cardiolipin (aCL) antibodies at pSLE diagnosis and 3 had aCL antibodies at endocarditis diagnosis. Four patients were on treatment with steroids, 2 were on hydroxychloroquine, and 2 were treatment-naive. One patient was treated with Cyclophosphamide, Rituximab, and plasmapheresis for pulmonary hemorrhage. The mean SLEDAI at diagnosis of endocarditis was 10.5 +/- 6.4. Two patients had baseline echocardiograms at the time of pSLE diagnosis; both were normal.

Two patients had LSE, with involvement of mitral and aortic valves on echocardiogram. Two patients had isolated IE without LSE, more commonly involving the tricuspid valve. Two patients were on antibiotics prior to obtaining blood cultures so overlying IE could not be ruled out. The only other concurrent clinical manifestation of lupus was arthritis in 2 patients.

No patients had an increase or change in immunosuppression specifically for treatment of LSE. Five patients received prophylactic anti-coagulation. One patient was placed on subacute bacterial endocarditis prophylaxis. The most severe complications were pulmonary embolus (one definite, one possible), distal emboli and Roth spots (2 patients).

Conclusion:

This is the largest case series of endocarditis in pSLE to date. Our data demonstrate endocarditis (LSE and/or IE) is more frequently diagnosed early in disease course, often in conjunction with other end-organ manifestations. As pSLE can have a more severe disease course than adult-onset SLE, our data suggest that endocarditis occurs in patients whose disease is either newly diagnosed or not well-controlled. Obtaining an echocardiogram at pSLE diagnosis with annual echocardiograms for surveillance may be useful. Anti-phospholipid antibodies play a role in LSE and should also be monitored.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/libman-sacks-endocarditis-in-pediatric-systemic-lupus-erythematosus-clinical-features-and-complications/