title: [Clinicopathological features of neuropathy associated with IgG4-related disease].
abstract: The newly recognized entity IgG4-related disease (IgG4-RD) is characterized by an elevated IgG4 serum concentration, swelling of organ, and tissue infiltration by IgG4-positive plasma cells with fibrosis. IgG4-RD has been reported in various organs. In the field of neurology, hypophysitis and hypertrophic pachymeningitis have been known to be related to IgG4-RD, while reported patients with neuropathy manifesting features compatible to IgG4-RD. The features of IgG4-related neuropathy are characterized by sensory-motor neuropathy, mononeuritis multiplex pattern, and predominant involvement of distal portions of the lower extremities. In the sural nerve biopsy specimens, fibrosis and IgG4-positive plasma cell infiltration in the epineurium and decreased myelinated fiber density due to axonal degeneration were observed. IgG4-RD should be considered as the differential diagnosis of neuropathy.
authors: Department of Neurology, Nagoya University Graduate School of Medicine.
authors: Ken
authors: K
authors: Ohyama
authors: Haruki
authors: H
authors: Koike
authors: Mie
authors: M
authors: Takahashi
authors: Yuichi
authors: Y
authors: Kawagashira
authors: Masahiro
authors: M
authors: Iijima
authors: Gen
authors: G
authors: Sobue
mesh_terms: D001369:Axons / Q000473:pathology; D005355:Fibrosis; D006801:Humans; D006984:Hypertrophy; D007074:Immunoglobulin G / Q000097:blood*; D008581:Meningitis / Q000276:immunology / Q000473:pathology; D020422:Mononeuropathies; D009410:Nerve Degeneration / Q000473:pathology; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D010525:Peripheral Nerves / Q000473:pathology; D010900:Pituitary Diseases / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology
chemical_list: D007074:Immunoglobulin G