Current Allergy and Asthma Reports

, Volume 7, Issue 5, pp 323-330

First online:

Hereditary immunologic disorders caused by pyrin and cryopyrin

  • Hal M. HoffmanAffiliated withDivision of Rheumatology, Allergy, and Immunology, University of California at San Diego Medical School Email author 

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A new family of hereditary immunologic disorders known as the autoinflammatory diseases involves dysregulation of the innate immune system. Elucidation of the genetic basis of these disorders has resulted in improved understanding of the disease pathophysiology of systemic and tissue inflammation, and has also revealed novel nonpathologic innate immune mechanisms. These advances have also resulted in direct improvement in diagnosis and therapy for autoinflammatory disorders such as the cryopyrinopathies and familial Mediterranean fever and have implications for more common inflammatory diseases.