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Cytokine Storm Syndrome pp 407–421Cite as

The Intersections of Autoinflammation and Cytokine Storm

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Abstract

Autoinflammation is a category of human immune dysregulation best exemplified by inborn errors in genes important for innate immunity. Though overproduction of cytokines is a mechanistic feature of most autoinflammatory diseases, only a few have been associated with the phenotype of Cytokine Storm. Rarely, the most severe presentations of some canonical autoinflammatory diseases have featured cytokine storm. Innate immune activation is also part of the pathogenesis of canonical cytokine storm disorders, like hemophagocytic lymphohistiocytosis. More recently, biomarker, mechanistic, and early treatment studies in patients with Still’s disease, or with NLRC4 or XIAP mutations, have placed the inflammasome-activated cytokine IL-18 at the center of the intersection of autoinflammation and cytokine storm.

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  1. RK Mellon Institute for Pediatric Research/Pediatric Rheumatology, University of Pittsburgh/UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Scott W. Canna

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  1. UAB School of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA

    Randy Q. Cron

  2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Edward M. Behrens

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Canna, S.W. (2019). The Intersections of Autoinflammation and Cytokine Storm. In: Cron, R., Behrens, E. (eds) Cytokine Storm Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-22094-5_24

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