RT Book, Section
A1 Gottlieb, Peter A.
A2 Jameson, J. Larry
SR Print(0)
ID 1141276340
T1 Autoimmune Polyendocrine Syndromes
T2 Harrison's Endocrinology, 4e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9781259835728
LK accessbiomedicalscience.mhmedical.com/content.aspx?aid=1141276340
RD 2024/04/24
AB Polyglandular  deficiency  syndromes  have  been  given  many  different  names,  reflecting  the  wide  spectrum  of  disorders  that  have  been  associated  with  these  syndromes  and  the  heterogeneity  of  their  clinical  presentations.  The  name  used  in  this  chapter  for  this  group  of  disorders  is  autoimmune  polyendocrine  syndrome  (APS).  In  general,  these  disorders  are  divided  into  two  major  categories,  APS  type  1  (APS-1)  and  APS  type  2  (APS-2).  Some  groups  have  further  subdivided  APS-2  into  APS  type  3  (APS-3)  and  APS  type  4  (APS-4)  depending  on  the  type  of  autoimmunity  involved.  For  the  most  part,  this  additional  classification  does  not  clarify  our  understanding  of  disease  pathogenesis  or  prevention  of  complications  in  individual  patients.  Importantly,  there  are  many  nonendocrine  disease  associations  included  in  these  syndromes,  suggesting  that  although  the  underlying  autoimmune  disorder  predominantly  involves  endocrine  targets,  it  does  not  exclude  other  tissues.  The  disease  associations  found  in  APS-1  and  APS-2  are  summarized  in  Table  30-1.  Understanding  these  syndromes  and  their  disease  manifestations  can  lead  to  early  diagnosis  and  treatment  of  additional  disorders  in  patients  and  their  family  members.