PMID- 29377160
OWN - NLM
STAT- MEDLINE
DCOM- 20190311
LR  - 20190311
IS  - 1749-6632 (Electronic)
IS  - 0077-8923 (Print)
IS  - 0077-8923 (Linking)
VI  - 1413
IP  - 1
DP  - 2018 Feb
TI  - IgG4-mediated autoimmune diseases: a niche of antibody-mediated disorders.
PG  - 92-103
LID - 10.1111/nyas.13561 [doi]
AB  - Immunoglobulin 4 (IgG4) is one of four human IgG subclasses and has several 
      unique functional characteristics. It exhibits low affinity for complement and 
      for most Fc receptors. It furthermore has generally high affinity for its 
      antigen, with binding occurring in a monovalent fashion, as IgG4 can exchange 
      Fab-arms with other IgG4 molecules. Because of these characteristics, IgG4 is 
      believed to block its targets and prevent inflammation, which, depending on the 
      setting, can have a protective or pathogenic effect. One example of IgG4 
      pathogenicity is muscle-specific kinase (MuSK) myasthenia gravis (MG), in which 
      patients develop IgG4 MuSK autoantibodies, resulting in muscle weakness. As a 
      consequence of the distinct IgG4 characteristics, the pathomechanism of MuSK MG 
      is very different from IgG1-and IgG3-mediated autoimmune diseases, such as 
      acetylcholine receptor MG. In recent years, new autoantibodies in a spectrum of 
      autoimmune diseases have been discovered. Interestingly, some were found to be 
      predominantly IgG4. These IgG4-mediated autoimmune diseases share many 
      pathomechanistic aspects with MuSK MG, suggesting that IgG4-mediated autoimmunity 
      forms a separate niche among the antibody-mediated disorders. In this review, we 
      summarize the group of IgG4-mediated autoimmune diseases, discuss the role of 
      IgG4 in MuSK MG, and highlight interesting future research questions for 
      IgG4-mediated autoimmunity.
CI  - (c) 2018 The Authors. Annals of the New York Academy of Sciences published by Wiley 
      Periodicals, Inc. on behalf of New York Academy of Sciences.
FAU - Huijbers, Maartje G
AU  - Huijbers MG
AD  - Departments of Neurology, Leiden University Medical Centre, Leiden, the 
      Netherlands.
AD  - Department of Human Genetics, Leiden University Medical Centre, Leiden, the 
      Netherlands.
FAU - Plomp, Jaap J
AU  - Plomp JJ
AD  - Departments of Neurology, Leiden University Medical Centre, Leiden, the 
      Netherlands.
FAU - van der Maarel, Silvere M
AU  - van der Maarel SM
AD  - Department of Human Genetics, Leiden University Medical Centre, Leiden, the 
      Netherlands.
FAU - Verschuuren, Jan J
AU  - Verschuuren JJ
AD  - Departments of Neurology, Leiden University Medical Centre, Leiden, the 
      Netherlands.
LA  - eng
GR  - R21 NS088723/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Review
DEP - 20180128
PL  - United States
TA  - Ann N Y Acad Sci
JT  - Annals of the New York Academy of Sciences
JID - 7506858
RN  - 0 (Autoantibodies)
RN  - 0 (Autoantigens)
RN  - 0 (Immunoglobulin G)
RN  - 0 (Receptors, Cholinergic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Autoantibodies/*immunology
MH  - Autoantigens/*immunology
MH  - Autoimmunity/immunology
MH  - Humans
MH  - Immunoglobulin G/classification/*immunology
MH  - Muscle Weakness/pathology
MH  - Myasthenia Gravis/*immunology
MH  - Neuromuscular Junction/metabolism
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
PMC - PMC5801142
MID - NIHMS918108
OTO - NOTNLM
OT  - IgG4
OT  - MuSK
OT  - autoimmunity
OT  - myasthenia gravis
OT  - neuromuscular junction
COIS- Competing interests The authors declare no competing interests.
EDAT- 2018/01/30 06:00
MHDA- 2019/03/12 06:00
PMCR- 2019/02/01
CRDT- 2018/01/30 06:00
PHST- 2017/08/10 00:00 [received]
PHST- 2017/10/19 00:00 [revised]
PHST- 2017/10/30 00:00 [accepted]
PHST- 2018/01/30 06:00 [pubmed]
PHST- 2019/03/12 06:00 [medline]
PHST- 2018/01/30 06:00 [entrez]
PHST- 2019/02/01 00:00 [pmc-release]
AID - 10.1111/nyas.13561 [doi]
PST - ppublish
SO  - Ann N Y Acad Sci. 2018 Feb;1413(1):92-103. doi: 10.1111/nyas.13561. Epub 2018 Jan 
      28.