IL-1 blockade in autoinflammatory syndromes.

  title={IL-1 blockade in autoinflammatory syndromes.},
  author={Adriana Almeida de Jesus and Raphaela T Goldbach-Mansky},
  journal={Annual review of medicine},
Monogenic autoinflammatory syndromes present with excessive systemic inflammation including fever, rashes, arthritis, and organ-specific inflammation and are caused by defects in single genes encoding proteins that regulate innate inflammatory pathways. Pathogenic variants in two interleukin-1 (IL-1)-regulating genes, NLRP3 and IL1RN, cause two severe and early-onset autoinflammatory syndromes, CAPS (cryopyrin associated periodic syndromes) and DIRA (deficiency of IL-1 receptor antagonist). The… CONTINUE READING


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A locus for familial cold urticaria maps to distal chromosome 1q: familial cold urticaria and Muckle-Wells-syndrome are probably allelic

  • M Jung, B Ross, TF Wienker
  • Am. J. Hum. Genet. Suppl
  • 1996
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