Immunoglobulin G4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD can occur in various organs, including the pancreas, lacrimal gland, salivary gland, thyroid, lung, bile duct, liver, gastrointestinal tract, kidney, prostate, retroperitoneum, arteries, lymph nodes, skin, and breast. Steroid therapy is often effective. In the field of neurology, pachymeningitis (IgG4-related pachymeningitis) and hypophysitis (IgG4-related hypophysitis) are known to be related to IgG4-RD. Recently, a few papers have described the involvement of peripheral nerves in IgG4-RD. Here, we describe the concept of IgG4-RD and highlight the involvement of the central and peripheral nervous systems in IgG4-RD.
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