Search

Monogenic autoinflammatory syndromes comprise disorders caused by mutations of different genes encoding for proteins that play a major role in the inflammatory response. They are characterized by seemingly unprovoked inflammatory episodes lacking high concentrations of autoantibodies or autoreactive T cells. The clinical spectrum of these syndromes are extremely variable and is represented by: recurrent fever episodes of variable duration (periodic/recurrent fever), skin lesions (cryopyrinopathies), noncaseating granulomas (granulomatous disorders) and, rarely, by sterile pyogen abscesses (pyogenic disorders). This paper represents a briefly presentation of these disorders. [ABSTRACT FROM AUTHOR]