Autoinflammatorische Syndrome

Translated title of the contribution: Autoinflammatory syndromes

P. Lamprecht*, W. L. Gross

*Corresponding author for this work
5 Citations (Scopus)


In its strict sense, the term "autoinflammatory syndromes" comprises the hereditary periodic fever syndromes (HPF), which are caused bymutations of pattern-recognition receptors (PRR) and perturbations of thecytokine balance. These include the crypyrinopathies, familial Mediterraneanfever, TNF-receptor associated periodic fever syndrome (TRAPS), hyper-IgD andperiodic syndrome (HIDS), pyogenic sterile arthritis, pyoderma gangrenosum andacne (PAPA) syndrome, NALP12-HPF, and the Blau syndrome. The diseases arecharacterized by spontaneous activation of cells of the innate immunity in theabsence of ligands. Autoantibodies are usually not found. HPF clinically presentwith recurrent fever episodes and inflammation, especially of serosal andsynovial interfaces and the skin. Intriguingly, PRR-mediated autoinflammtorymechanisms also play a role in a number of chronic inflammatory and autoimmunediseases.

Translated title of the contributionAutoinflammatory syndromes
Original languageGerman
Issue number6
Pages (from-to)676-684
Number of pages9
Publication statusPublished - 06.2009

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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