Abstract
The periodic fever syndrome constitutes a group of disorders characterized by recurrent attacks of fever accompanied with other inflammatory symptoms typical for any given unit. Attacks are separated by asymptomatic period.
The periodic fever syndromes are disorders that lead to the abnormal regulation and activation of inflammation. The same cause has a larger group of so-called autoinflammatory diseases that present with fever and other clinical symptoms, affecting mostly serous membranes, joints, skin and eyes.
With ongoing research, a group of autoinflammatory diseases still expanding with new, well- defined units including a classical monogenic hereditary periodic fever syndromes , followed by a group of idiopathic syndromes without known molecular causes, but with a clear clinical definition , as well as the pyogenic and granulomatous diseases and autoinflammatory diseases of the skin and bones.