RT Book, Section
A1 Stone, John H.
A2 Imboden, John B.
A2 Hellmann, David B.
A2 Stone, John H.
SR Print(0)
ID 57276392
T1 Chapter 71. IgG4-Related Disease
T2 CURRENT Diagnosis & Treatment: Rheumatology, 3e
YR 2013
FD 2013
PB The McGraw-Hill Companies
PP New York, NY
SN 978-0-07-163805-0
LK accessmedicine.mhmedical.com/content.aspx?aid=57276392
RD 2024/04/19
AB IgG4-related  disease  (IgG4-RD)  is  a  potentially  multi-organ  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;  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%).  One  explanation  for  the  finding  of  normal  serum  IgG4  concentrations  may  be  the  prozone  phenomenon,  a  spuriously  low  result  reported  sometimes  when  the  analyte  (IgG4)  is  actually  present  at  exceptionally  high  concentrations.  This  problem  may  be  circumvented  by  diluting  test  samples  sufficiently.Serum  IgG4  concentrations  sometimes  correlate  with  disease  activity.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.