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Autoinflammatory diseases 

Autoinflammatory diseases
Chapter:
Autoinflammatory diseases
Source:
Oxford Textbook of Rheumatology (4 ed.)
Author(s):

Helen J. Lachmann

and Philip N. Hawkins

DOI:
10.1093/med/9780199642489.003.0164

The autoinflammatory diseases are disorders of innate immunity which result in chronic or recurrent multisystem inflammatory disease. This chapter concentrates on the currently known inherited periodic fever syndromes. These rare genetic diseases have resulted in major advances in the understanding of innate immunity in general and the regulation of interleukin (IL)-1 in particular. It now seems clear that autoinflammatory conditions are disorders of the regulation of IL-1 and NFκ‎B. The treatment and prognosis of these diseases have been radically improved by the availability of cytokine-blocking agents.

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