Published: 21 September 2022
Author(s): Anouk Le Goueff, Guillaume Smits, Mélanie Delaunoy, Isabelle Vandernoot, Frédéric Vandergheynst
Section: Original article

Autoinflammatory diseases (AIDs) or hereditary recurrent fevers (HRFs) are a group of rare and mostly monogenic illnesses with mendelian heredity. They are caused by a class IV or class V gene variant in a gene responsible for immune regulation, thus leading to uncontrolled activation of the innate immune system. Affected patients present with recurrent episodes of fever and elevated acute phase reactants, as well as organ-specific symptoms [1–3].

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