Autoinflammatory syndromes (overview)

Group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune and non-immunodeficient in cause. The term "autoinflammatory diseases" includes the hereditary periodic fever syndromes (HPF), which are induced by mutations of pattern-recognition receptors (PRR) and disturbances of cytokine generation and balance.

Distinguished are:

• Hereditary periodic fever syndromes

• other hereditary and non-hereditary idiopathic fever syndromes such as:
  • Still Syndrome
  • Adult onset Still's disease (AOSD)
  • Schnitzler syndrome (urticarial exanthema)
  • SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis)
  • PASH syndrome (pyoderma gangraenosum, acne, suppurative hidradenitis)
  • PAPA syndrome (pyoderma gangraenosum, acne, pyogenic arthritis)
• various. autoimmune syndromes as UAW

• Initially, the term "autoinflammatory syndromes" was coined for a small group of familial fever syndromes (see below Fever syndromes, hereditary, periodic ).
• This term has since been expanded to include an increasing number of acquired disorders.
• In 2010, a drug-induced autoinflammatory syndrome was described, the "autoinflammatory syndrome induced by adjuvants (ASIA)". Gulf War Syndrome" also belongs to this group of syndromes.
• Such reactions are interpreted as adverse drug reactions(ADRs) occurring as a result of vaccination (see also AEFI ) .

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1. Braun-Falco M et al (2011) Skin involvement in autoinflammatory syndromes. SDDG 9: 232 - 245
2. Israeli E (2012) Gulf War syndrome as a part of the autoimmune (autoinflammatory) syndrome induced by adjuvant (ASIA). Lupus 21:190-194.