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Auto-inflammatory syndromes are defined as conditions caused by an exaggerated innate immune system response, resulting in episodes of spontaneous inflammation affecting multiple organs. The prototypical auto-inflammatory disorders are associated with periodic febrile episodes. Auto-inflammatory syndromes now include polygenic diseases, such as Behcet's syndrome and Still's disease; however, the best characterized auto-inflammatory diseases are relatively rare, but florid conditions arising from mutations in single genes. The prevalence of each auto-inflammatory disease varies depending on ethnic background.

pediatric rheumatology online journal

Prevalence of polymorphisms of the genes responsible for auto-inflammatory diseases among 202 patients with recurrent fever in a rheumatology institute in Japan