title: The expanding field of IgG4-mediated neurological autoimmune disorders.
abstract: At least 13 different disease entities affecting the central nervous system, peripheral nervous system and connective tissue of the skin or kidneys are associated with immunoglobulin G4 (IgG4) immune reactivity. IgG4 has always been considered a benign, non-inflammatory subclass of IgG, in contrast to the well-known complement-activating pro-inflammatory IgG1 subclass. A comprehensive review of these IgG4 autoimmune disorders reveals striking similarities in epitope binding and human leukocyte antigen (HLA) associations. Mechanical interference of extracellular ligand-receptor interactions by the associated IgG4 antibodies seems to be the common/converging disease mechanism in these disorders.
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.|Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: M G
authors.initials: MG
authors.lastname: Huijbers
authors.affiliation: Department of Neurology, Hospital Santa Creu I Sant Pau, Barcelona, Spain.
authors.forename: L A
authors.identifier: http://orcid.org/0000-0002-4289-8264
authors.initials: LA
authors.lastname: Querol
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: E H
authors.initials: EH
authors.lastname: Niks
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J J
authors.initials: JJ
authors.lastname: Plomp
authors.affiliation: Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: S M
authors.initials: SM
authors.lastname: van der Maarel
authors.affiliation: Department of Neurology, Hospital Santa Creu I Sant Pau, Barcelona, Spain.
authors.forename: F
authors.initials: F
authors.lastname: Graus
authors.affiliation: Department of Neurology, Hospital Santa Creu I Sant Pau, Barcelona, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Dalmau
authors.affiliation: Department of Neurology, Hospital Santa Creu I Sant Pau, Barcelona, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Illa
authors.affiliation: Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J J
authors.identifier: http://orcid.org/0000-0002-4572-1501
authors.initials: JJ
authors.lastname: Verschuuren
mesh_terms: D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*
chemical_list: D007074:Immunoglobulin G
keywords: CIDP; IgG4; Iglon5; Lgi1; MuSK myasthenia gravis; autoimmunity; membranous nephropathy; neurofascin; pemphigus