RT Book, Section
A1 Hashkes, Philip J.
A2 Kline, Mark W.
SR Print(0)
ID 1182932732
T1 Autoinflammatory Disorders
T2 Rudolph's Pediatrics, 23e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259588594
LK accesspediatrics.mhmedical.com/content.aspx?aid=1182932732
RD 2024/04/19
AB Autoinflammatory  syndromes,  formerly  known  as  periodic  fever  syndromes,  are  defined  as  recurrent  attacks  of  often  unprovoked  systemic  inflammation  that  are  related  to  a  lack  of  adequate  regulation  of  the  innate  immune  system.  Unlike  autoimmune  diseases,  these  conditions  are  not  generally  marked  by  autoantibodies  or  autoreactive  T  cells.  Many  of  these  syndromes  have  a  genetic  etiology  (Table  206-1).  The  pathogenesis  of  a  large  proportion  of  the  major  syndromes  involves  the  excessive  production  and  activity  of  interleukin  (IL)-1β  (Fig.  206-1).  However,  other  immune  mechanisms  are  also  implicated  in  the  pathogenesis  of  these  diseases.  The  conditions  are  no  longer  known  as  periodic  fever  syndromes  because  most  are  not  truly  periodic  and  fever  is  not  a  necessary  feature.