PMID- 31852351
OWN - NLM
STAT- MEDLINE
DCOM- 20201106
LR  - 20201106
IS  - 1439-7609 (Electronic)
IS  - 1439-7595 (Linking)
VI  - 30
IP  - 4
DP  - 2020 Jul
TI  - IgG4-related disease: How to place it in the spectrum of immune-mediated and 
      rheumatologic disorders?
PG  - 609-616
LID - 10.1080/14397595.2019.1705537 [doi]
AB  - IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder 
      that results from massive expansion of polyclonal IgG4-switched B and/or plasma 
      cells. It can virtually affect all organs and its diagnosis relies on clinical, 
      serological and histopathological criteria. The role of autoimmunity and adaptive 
      immune system in IgG4-RD is reflected in plasmablast differentiation, germinal 
      center formation and IgG4 production induced by CD4+ cells expressing CD40 
      ligand. IgG4-RD has been considered to be a Th2/Treg-driven disorder, but 
      follicular helper T cells are important in driving the IgG subclass switch. 
      Prompt clinical responses to rituximab, human leukocyte antigen (HLA) 
      associations and the presence of autoantibodies also point to the importance of 
      adaptive immune system. However, innate immunity may induce storiform fibrosis 
      through T-cell independent responses as a consequence of toll-like receptors 
      activation by microbe-and damage-associated molecular patterns, while macrophages 
      and basophils also appear to have a significant role in IgG4-RD pathogenesis. 
      Allergic mechanisms may drive IgG4-RD, but only a subgroup has elevated IgE serum 
      levels and peripheral eosinophilia. Finally, the 2012 revised Chapel Hill 
      Consensus Conference nomenclature pointed IgG4-RD as a cause of large-vessel 
      vasculitis. This review aims to discuss how to place IgG4-RD in the spectrum of 
      immune-mediated and rheumatologic disorders.
FAU - Borges, Tiago
AU  - Borges T
AUID- ORCID: 0000-0002-8271-9865
AD  - Department of Internal Medicine, Trofa Saude Hospital Privado de Gaia, Vila Nova 
      de Gaia, Portugal.
FAU - Silva, Sergio
AU  - Silva S
AD  - Department of Internal Medicine, Trofa Saude Hospital Privado de Gaia, Vila Nova 
      de Gaia, Portugal.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20200104
PL  - England
TA  - Mod Rheumatol
JT  - Modern rheumatology
JID - 100959226
RN  - 0 (Immunologic Factors)
RN  - 4F4X42SYQ6 (Rituximab)
SB  - IM
MH  - Consensus
MH  - Humans
MH  - Immunoglobulin G4-Related Disease/classification/drug 
      therapy/*immunology/pathology
MH  - Immunologic Factors/therapeutic use
MH  - Lymphocytes/immunology
MH  - Rituximab/therapeutic use
OTO - NOTNLM
OT  - IgG4
OT  - allergy
OT  - autoimmune
OT  - autoinflammatory
OT  - vasculitis
EDAT- 2019/12/20 06:00
MHDA- 2020/11/11 06:00
CRDT- 2019/12/20 06:00
PHST- 2019/12/20 06:00 [pubmed]
PHST- 2020/11/11 06:00 [medline]
PHST- 2019/12/20 06:00 [entrez]
AID - 10.1080/14397595.2019.1705537 [doi]
PST - ppublish
SO  - Mod Rheumatol. 2020 Jul;30(4):609-616. doi: 10.1080/14397595.2019.1705537. Epub 
      2020 Jan 4.