Article
Monogenic autoinflammatory diseases: disorders of amplified danger sensing and cytokine dysregulation.
Rheumatic diseases clinics of North America - 1 Nov 2013
Sanchez Gina A Montealegre, de Jesus Adriana Almeida, Goldbach-Mansky Raphaela
Abstract excerpt
The pathogenesis of monogenic autoinflammatory diseases converges on the presence of exaggerated immune responses that are triggered through activation of altered pattern recognition receptor (PRR) pathways and result in cytokine/chemokine amplification loops and the inflammatory clinical phenotype seen in autoinflammatory patients. The PRR response can be triggered by accumulation of metabolites, by mutations in...
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