TY  - CHAP
M1  - Book, Section
TI  - IgG4-Related Disease
A1  - Stone, John H.
A2  - Stone, John H.
PY  - 2021
T2  - Current Diagnosis & Treatment: Rheumatology, 4e
AB  - ESSENTIALS  OF  DIAGNOSISIgG4-related  disease  (IgG4-RD)  is  a  multiorgan  disease  with  highly  characteristic  pathology  findings  and  immunostaining  characteristics  across  involved  tissues.Organ  system  involvement  may  be  confined  to  single  organs  but  in  many  cases  evolves  over  months  to  years  to  involve  multiple  organs  in  either  a  sequential  or  simultaneous  fashion.Commonly  involved  organs  include  the  salivary  glands  (submandibular,  parotid);  the  orbits  and  lacrimal  glands;  the  thyroid  gland;  the  lymph  nodes;  the  thoracic  and  abdominal  aorta;  the  mediastinum,  retroperitoneum,  and  mesentery;  and  the  lungs,  biliary  tree,  pancreas,  and  kidneys.IgG4-RD  has  also  been  reported  in  the  pachymeninges,  the  skin,  and  the  prostate  gland.Serum  IgG4  concentrations  are  elevated  in  most  patients  (approximately  70%).  In  patients  with  exceptionally  high  serum  IgG4  concentrations—usually  those  with  multiorgan  disease—the  prozone  phenomenon  can  lead  to  a  spuriously  low  result.  This  problem  may  be  circumvented  by  diluting  test  samples  sufficiently.If  elevated  at  the  time  of  diagnosis,  serum  IgG4  concentrations  are  generally  a  good  biomarker.  Other  useful  biomarkers  in  individual  patients  include  IgG1,  IgE,  and  complement  levels  (C3  and  C4).Histopathologic  hallmarks:  lymphoplasmacytic  tissue  infiltrate,  storiform  fibrosis,  obliterative  phlebitis,  germinal  center  formation,  and  mild  to  modest  tissue  eosinophilia.Immunostaining  characteristics:  A  high  percentage  of  plasma  cells  stain  positively  for  IgG4.  
SN  - 
PB  - McGraw Hill
CY  - New York, NY
Y2  - 2024/04/18
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1180198713
ER  -