Fatal catastrophic antiphospholipid syndrome at presentation of juvenile lupus

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome, extremely uncommon in
the pediatric age group, characterized by multiple site thrombosis involving small, medium, and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We report the case of an 8-year-old girl presenting with fever, lymphadenopathy, and pain abdomen with refractory mitral regurgitation diagnosed as systemic lupus erythematosus with CAPS. All three antiphospholipid antibodies positivity in high titers further confirmed the diagnosis. In spite of the early initiation of adequate triple therapy (anticoagulation, steroids, and cyclophosphamide), our patient succumbed. The report aims to incorporate a greater awareness among clinicians for timely diagnosis and treatment of this condition and throws light on the varying ways in which lupus can present in children.