Schnitzler Syndrome

Síndrome autoinflamatório adquirido
 
  • Major criteria (2)
    • Recurrent urticarial rash
    • Monoclonal gammopathy (IgM>>IgG) - without criteria for Waldenstrom disease
  • Minor criteria
    • recurrent fever attacks (>38ºC without any other cause)
    • changes in bone visible in imaging
    • neutrophilic dermal infiltrate
    • leukocytosis and elevated CRP
  • Also may have
    • arthragia or arthritis
    • lymphadenopathy
  • It was first described by Dr. Liliane Schnitzler in 1972.
  • Tratamento com bloqueio IL-1 (anakinra, canakinumab, rilonacept)