Autoinflammatory syndromes (overview)

Group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune in nature. The term "autoinflammatory diseases" includes hereditary diseases (e.g. hereditary periodic fever syndromes) induced by mutations of pattern-recognition receptors (PRRs) and disturbances of cytokine generation and balance (see immunodeficiencies primary/autoinflammatory diseases) but also non-hereditary acquired autoinflammatory diseases.

Distinguished between:

• Hereditary periodic fever syndromes, which are classified as primary immunodeficiencies/autoinflammatory diseases in the IUIS classification (2019)

and

• other hereditary and non-hereditary idiopathic fever syndromes, which are also grouped under the term "autoinflammatory syndromes":
  • Still syndrome
  • Adult onset Still's syndrome (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)

Initially, the term "autoinflammatory syndromes" was coined for a small group of familial fever syndromes(see below Fever syndromes, hereditary, periodic; see below. Immunodeficiency primary (autoinflammatory diseases). 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)." The "Gulf War Syndrome" also belongs to this acquired group of diseases.

Such reactions are interpreted as adverse drug reactions(ADR) occurring as a consequence of vaccination (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.