The inherited autoinflammatory syndrome: a decade of discovery.

  title={The inherited autoinflammatory syndrome: a decade of discovery.},
  author={Stephen Goldfinger},
  journal={Transactions of the American Clinical and Climatological Association},
The hereditary autoinflammatory diseases arise from mutations of genes regulating the innate immune system. These rare disorders are well characterized, both clinically and in terms of their molecular pathogenesis. The recurrent attacks of febrile polyserositis of Familial Mediterranean Fever (FMF) are due to defective pyrin, a protein that down-regulates inflammation. The Hyperimmunoglobulinemia D Syndrome (HIDS), which mimics FMF, results from a genetically conferred deficiency of mevalonate… CONTINUE READING
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