RT Book, Section
A1 Grandemange, Sylvie
A1 Kone-Paut, Isabelle
A1 Touitou, Isabelle
A2 Murray, Michael F.
A2 Babyatsky, Mark W.
A2 Giovanni, Monica A.
A2 Alkuraya, Fowzan S.
A2 Stewart, Douglas R.
SR Print(0)
ID 1102702400
T1 Familial Autoinflammatory Diseases
T2 Clinical Genomics: Practical Applications in Adult Patient Care
YR 2014
FD 2014
PB McGraw-Hill Education
PP New York, NY
SN 9780071622448
LK accessmedicine.mhmedical.com/content.aspx?aid=1102702400
RD 2024/04/17
AB Disease  summary:Autoinflammatory  diseases  (AIDs)  are  illnesses  of  the  innate  immune  system  without  high-titer  autoantibodies  or  antigen-specific  T  cells  in  contrast  to  autoimmune  diseases  that  relate  to  a  deficit  of  acquired  immunity.  These  diseases  are  characterized  by  recurrent  fever  and  systemic  inflammation,  and  the  main  complication  is  the  risk  of  generalized  or  renal  amyloidosis  in  untreated  patients.  Other  sporadic,  undefined,  or  complex  disorders  have  been  also  linked  to  the  AIDs  group.  This  review  will  focus  on  hereditary  recurrent  fevers  (HRFs).Hereditary  basis:Both  dominant  and  recessive  autosomal  transmission,  with  incomplete  penetranceDifferential  diagnosis:Nonhereditary  recurrent  fevers,  for  example,  PFAPA  syndrome  (Marshall  syndrome)