RT Book, Section
A1 Hashkes, Philip J.
A2 Rudolph, Colin D.
A2 Rudolph, Abraham M.
A2 Lister, George E.
A2 First, Lewis R.
A2 Gershon, Anne A.
SR Print(0)
ID 7019999
T1 Chapter 209. Autoinflammatory Disorders
T2 Rudolph's Pediatrics, 22e
YR 2011
FD 2011
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-149723-7
LK accesspediatrics.mhmedical.com/content.aspx?aid=7019999
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.1-3  Unlike  autoimmune  diseases,  these  conditions  are  not  generally  marked  by  autoantibodies  or  autoreactive  T  cells.  Many  of  these  syndromes  have  a  genetic  etiology3,4  (eTable  209.1)  involving  the  excessive  production  and  activity  of  interleukin  (IL)-1β.5  The  conditions  are  no  longer  known  as  periodic  fever  syndromes,  6-8  because  most  are  not  truly  periodic  nor  is  fever  a  necessary  feature.