In the past 10 years, auto inflammatory diseases have gained recognition as a distinct class of disorders that are characterized by recurrent episodes of systemic inflammation resulting in fevers, leukocyte-mediated tissue infiltration, and dysregulated cytokine production. The recent discovery of genetic determinants of this spectrum of disorders has resulted in new insights into these diseases. Unlike autoimmune diseases, autoinflammatory diseases are marked by the notable absences of autoantibodies or the critical role of self-reactive B and T lymphocytes.
Shinkai K, Kilcline C, Connolly MK, Frieden IJ. The Pyrin Family of Fever Genes: Unmasking Genetic Determinants of Autoinflammatory Disease. Arch Dermatol. 2005;141(2):242–247. doi:10.1001/archderm.141.2.242
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