Pediatric anti-phospholipid syndrome nephropathy: a rare differential diagnosis for acute kidney injury in children.

Abstract

Pediatric anti-phospholipid syndrome (APS) is a rarely diagnosed multisystem autoimmune inflammatory disorder resulting in recurrent vascular thrombosis with serious clinical implications. We highlight a case of an adolescent female who presented with Catastrophic APS (CAPS) in hypertensive emergency and end organ damage. She developed reduced kidney function, fluid overload and neurological manifestations. Neuroimaging and kidney biopsy confirmed micro-angiopathic thrombosis. Her blood results showed high titers of triple positive anti-phospholipid antibodies with negative serology for other immunological conditions. She was managed with dual anti-coagulation and immunosuppression and remains under pediatric subspecialist follow-up for ongoing multisystem involvement. APS should be considered as a differential diagnosis in children presenting with sepsis and multi-organ dysfunction. Pediatric focused diagnostic criteria and long-term management is needed to cater to needs of the pediatric population.