A novel presentation of childhood systemic lupus erythematosus in a 6-year-old child

Abstract

Childhood systemic lupus erythematosus (cSLE) is an autoimmune disorder, characterized by the formation of autoantibodies, that most often presents in children around the age of 12. Antiphospholipid antibody syndrome (APS) is a condition in which antiphospholipid antibodies produced by the patient cause the formation of thromboses. While reports estimate that 30%–40% of cSLE patients have antiphospholipid antibodies, only 4% develop symptomatic APS, with venous thromboses being more common than arterial. Overall, thrombosis has an incidence of about 0.1/10,000 in the pediatric population and usually only occurs in the setting of underlying disease or in relation to medical procedures. Here, we report a case of a 6-year-old female who presented with abdominal pain, arthralgia, fever, and right popliteal artery thrombus. Further testing revealed an abundance of autoantibodies including antinuclear, lupus anticoagulant, and anticardiolipin which resulted in a diagnosis of cSLE with associated APS. This presentation is novel due to both the young age of the patient at disease onset and the presence of concurrent cSLE and APS at initial diagnosis. Childhood systemic lupus erythematosus can be difficult for providers to recognize as it can present with varied nonspecific symptoms but being aware of the potential for onset in children significantly younger than the average will allow for early detection and potentially better outcomes.