Cardiac tamponade as the initial presentation of childhood systemic lupus erythematosus: A case report

Abstract

Systemic Lupus erythematosus (SLE) is an autoimmune disorder characterized by the proliferation of
autoantibodies and immune dysregulation resulting in damage to many body organs. Pediatric SLE
usually presents with fever, joint pain, rashes, and lupus nephritis. It is uncommon to have large
pericardial effusions in children with SLE and cardiac tamponade as the initial presentation of SLE is
even rarer.
An 11-year-old female presented to our Children Emergency Unit with fever and fast breathing for two
weeks, bilateral leg swelling of four days, and cough of two days duration. She was acutely ill,
tachypneic, and dyspneic with marked orthopnea, bilateral leg edema, and raised JVP. She was
tachycardic with a diffuse apex beat. Chest X-ray showed a large globular heart. 2D-Echocardiography
showed a large circumferential pericardial effusion with a dilated non-collapsing IVC and diastolic
collapse of the right ventricle. She had a pericardiotomy done and 650mls of serous pericardial fluid was
drained. The inner pericardium had a fibrinoid exudate with a “bread-and-butter” appearance. Pericardial
fluid cytology showed no malignant cells while pericardial biopsy showed suppurative granulomatous
inflammation. Antinuclear antibody (ANA) was strongly positive. The patient was managed with
corticosteroids, colchicine, and hydroxychloroquine, and has remained stable on follow-up.
While cardiac tamponade as an initial presenting complaint in SLE is rare, it is important that children
presenting with large pericardial effusions and tamponade be evaluated for rheumatologic disorders. This
can be crucial to revealing the correct diagnosis and instituting appropriate care.