TY  - CHAP
M1  - Book, Section
TI  - Familial Mediterranean Fever and Other Hereditary Autoinflammatory Diseases
A1  - Kastner, Daniel L.
A2  - Loscalzo, Joseph
A2  - Fauci, Anthony
A2  - Kasper, Dennis
A2  - Hauser, Stephen
A2  - Longo, Dan
A2  - Jameson, J. Larry
Y1  - 2022
N1  - 
T2  - Harrison's Principles of Internal Medicine, 21e
AB  - Familial  Mediterranean  fever  (FMF)  is  the  prototype  of  a  group  of  inherited  diseases  (Table  369-1)  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.  112).  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.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/23
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1198714777
ER  -