cytokine storm syndrome
Also written as: CSS — Cytokine Storm Syndrome
A life-threatening hyperinflammatory condition characterized by excessive immune activation and cytokine release, often complicating systemic juvenile arthritis.
Full Definition
Cytokine storm syndrome is a severe, potentially fatal hyperinflammatory state that occurs when the immune system releases excessive amounts of inflammatory cytokines. In pediatric rheumatology, it most commonly complicates systemic juvenile idiopathic arthritis but can also occur in other inflammatory conditions. The syndrome presents with high fever, organomegaly, cytopenias, hyperferritinemia, and elevated lactate dehydrogenase. Early recognition and aggressive immunosuppressive treatment are critical for patient survival. The condition shares clinical features with hemophagocytic lymphohistiocytosis and macrophage activation syndrome.
Usage
Usage note: Often used interchangeably with macrophage activation syndrome in pediatric rheumatology contexts, though subtle clinical distinctions may exist.
In Context
- "The patient's rapidly deteriorating condition with multiorgan dysfunction was consistent with cytokine storm syndrome." — Clinical case report
- "Prompt recognition of cytokine storm syndrome allowed for early intervention with high-dose corticosteroids." — Treatment protocol