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Still's Disease in the Constellation of Hyperinflammatory Syndromes: A Link with Kawasaki Disease?
Perrine Dusser1,2, Isabelle Koné-Paut1,2
1Paediatric Rheumatology Department, Université Paris-Saclay, APHP, Bicêtre Hospital, 94270 Le Kremlin-Bicêtre, France.
Insights
Still's disease and Kawasaki disease (KD) are cytokine storm syndromes. These conditions may represent a common spectrum influenced by genetic factors affecting the adaptive immune response.
Area of Science:
- Immunology
- Rheumatology
- Pediatrics
Background:
- Still's disease and Kawasaki disease (KD) are cytokine storm syndromes.
- Both are linked to excessive innate immune system activation.
Purpose of the Study:
- To explore the connection between Still's disease and KD.
- To propose a unifying concept for these distinct conditions.
Main Methods:
- Comparative analysis of clinical and pathophysiological features.
- Review of existing literature and theoretical framework.
Main Results:
- Significant clinical and pathophysiological similarities exist between Still's disease and KD.
- These diseases may exist on a spectrum of immune dysregulation.
Conclusions:
- Still's disease and KD share a common pathophysiological basis.
- Subtle differences in adaptive immune responses, potentially genetically influenced, may differentiate their clinical outcomes.
Abstract:
Still's disease and Kawasaki disease (KD) today belong to the group of cytokine storm syndromes, a pathophysiological set related to excessive activation of the innate immune response. We present here a personal vision of what can link these two diseases, taking up their concepts at their beginning. By their many clinical and physiopathological similarities, we conclude that they constitute a common spectrum whose fate is modified by subtle differences in terms of adaptive response that could, in part, be driven by genetic factors.
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