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, which are also assigned to immunodeficiencies group 7 in the IUIS classification (2019) and are named here as autoinflammatory diseases. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082388/
• other hereditary and non-hereditary idiopathic fever syndromes named as autoinflammatory 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)
  • Proteasome-associated autoinflammatory syndrome 2 (PRAAS2)
• versch. 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) that occur as a consequence of vaccinations (see also AEFI ).

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.