AUTHOR=Grados Aurélie , Ebbo Mikael , Piperoglou Christelle , Groh Matthieu , Regent Alexis , Samson Maxime , Terrier Benjamin , Loundou Anderson , Morel Nathalie , Audia Sylvain , Maurier François , Graveleau Julie , Hamidou Mohamed , Forestier Amandine , Palat Sylvain , Bernit Emmanuelle , Bonotte Bernard , Farnarier Catherine , Harlé Jean-Robert , Costedoat-Chalumeau Nathalie , Vély Frédéric , Schleinitz Nicolas 

TITLE=T Cell Polarization toward TH2/TFH2 and TH17/TFH17 in Patients with IgG4-Related Disease

JOURNAL=Frontiers in Immunology

VOLUME=Volume 8 - 2017

YEAR=2017

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2017.00235

DOI=10.3389/fimmu.2017.00235

ISSN=1664-3224

ABSTRACT=We examine and characterize subsets of circulating lymphocytes in untreated patients with active
IgG4-related disease (IgG4-RD). Twenty eight consecutive patients with proven IgG4-RD were
included in a prospective, multicentric study. Lymphocytes subsets were analyzed by flow cytometry,
with analysis of TH1/TH2/TH17, TFH cells and cytokine release by peripheral blood mononuclear cells.
Results were compared with healthy controls and with patients with primary Sjogren syndrome.
Patients with IgG4-RD were characterized by an increase in circulating T regulatory cells, TH2, TH17
and the CD4+CXCR5+PD1+ TFH subset. Accordingly, cytokine release was associated with increased
levels of IL-10 and IL-4. TFH increase was characterized by specific expansion of TFH2 (CCR6-
CXCR3-), and to a lesser extend TFH17 (CCR6+CXCR3-) cells. The CD4+CXCR5+PD1+ TFH cells
normalized under treatment.
We conclude that the functional orientation of circulating T cells towards a TH2/TFH2 and TH17/TFH17
profile is implicated in the pathophysiology of IgG4-RD. New treatment approaches should focus on
targeting specific T cell changes in patients with IgG4-RD.□