Macrophage activation syndrome during pregnancy as a fatal complication of systemic idiopathic arthritis

According to the current concept, juvenile arthritis with a systemic onset (systemic juvenile idiopathic arthritis, sJIA) and adult­onset Still's disease (ASD) are considered as rare autoinflammatory diseases, with the underlying immunopathogenesis being based on the mechanisms bridging both of such diseases. Despite they develop in childhood and young age, the issues of reproduction occurring during these pathologies virtually unexplored. The article presents a clinical case of maternal death in a 22-year-old primiparous woman and analyzes the tactics of sJIA management, which was complicated by pregnancy-related macrophage activation syndrome.