Neurologic Features of Immunoglobulin G4-Related Disease

Rheum Dis Clin North Am. 2017 Nov;43(4):621-631. doi: 10.1016/j.rdc.2017.06.010.


Immunoglobulin G4-related disease (IgG4-RD) can involve nearly any organ system, including the central and peripheral nervous systems. IgG4 antibodies are not known to play a primary etiologic role in disease. IgG4-RD must be distinguished from a growing number of immune-mediated conditions in which IgG4 autoantibodies contribute directly to pathophysiology. The most common neurologic features of IgG4-RD result from disease in the orbits, pachymeninges, and substance of the pituitary gland and stalk, as well as a perineuropathy that can involve peripheral or cranial nerves. Disease affecting the brain parenchyma is rare but reported.

Keywords: Hypophysitis; Immunoglobulin G4; Meningeal inflammation; Multiorgan disease; Orbital myositis; Orbital pseudo tumor.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoimmune Diseases of the Nervous System / diagnosis*
  • Autoimmune Diseases of the Nervous System / etiology*
  • Autoimmune Diseases of the Nervous System / therapy
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin G*


  • Glucocorticoids
  • Immunoglobulin G