RT Book, Section
A1 Kastner, Daniel L.
A2 Jameson, J. Larry
A2 Fauci, Anthony S.
A2 Kasper, Dennis L.
A2 Hauser, Stephen L.
A2 Longo, Dan L.
A2 Loscalzo, Joseph
SR Print(0)
ID 1159156357
T1 Familial Mediterranean Fever and Other Hereditary Autoinflammatory Diseases
T2 Harrison's Principles of Internal Medicine, 20e
YR 2018
FD 2018
PB McGraw-Hill Education
PP New York, NY
SN 9781259644016
LK accessmedicine.mhmedical.com/content.aspx?aid=1159156357
RD 2024/04/24
AB Familial  Mediterranean  fever  (FMF)  is  the  prototype  of  a  group  of  inherited  diseases  (Table  362-1)  that  are  characterized  by  recurrent  episodes  of  fever  with  serosal,  synovial,  or  cutaneous  inflammation  and,  in  some  individuals,  the  eventual  development  of  systemic  AA  amyloidosis  (Chap.  108).  Because  of  the  relative  infrequency  of  high-titer  autoantibodies  or  antigen-specific  T  cells,  the  term  autoinflammatory  has  been  proposed  to  describe  these  disorders,  rather  than  autoimmune.  The  innate  immune  system,  with  its  myeloid  effector  cells  and  germline  receptors  for  pathogen-associated  molecular  patterns  and  endogenous  danger  signals,  plays  a  predominant  role  in  the  pathogenesis  of  the  autoinflammatory  diseases.  Although  the  hereditary  recurrent  fevers  comprise  a  major  category  of  the  autoinflammatory  diseases,  other  inherited  disorders  of  inflammation  in  which  recurrent  fever  plays  a  less  prominent  role  are  now  also  considered  to  be  autoinflammatory.