Neurologic Features of Immunoglobulin G4-Related Disease

Mahmoud AbdelRazek; John H Stone

doi:10.1016/j.rdc.2017.06.010

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.

Authors

Mahmoud AbdelRazek¹, John H Stone²

Affiliations

¹ Department of Neurology, Mayo clinic, 200 First Street. SouthWest Rochester, MN 55905, USA.
² Rheumatology Unit, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. Electronic address: jhstone@mgh.harvard.edu.

PMID: 29061247
DOI: 10.1016/j.rdc.2017.06.010

Abstract

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*

Substances

Glucocorticoids
Immunoglobulin G