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Neuro-ophthalmic complications of IgG4-related disease

Chwalisz, Bart K.a; Stone, John H.b

Current Opinion in Ophthalmology: November 2018 - Volume 29 - Issue 6 - p 485–494
doi: 10.1097/ICU.0000000000000523
NEURO-OPHTHALMOLOGY: Edited by Dean M. Cestari

Purpose of review IgG4-related disease (IgG4-RD) is increasingly recognized as a fibroinflammatory disease with a plethora of organ-specific manifestations but a particular predilection for head and neck tissues, including the nervous system. This review discusses general features and organ-specific presentations of IgG4-RD as well as treatment considerations, particularly emphasizing features of neuro-ophthalmic interest.

Recent findings IgG4-RD is emerging as a common cause of several fibroinflammatory disorders in the head and neck that were previously considered idiopathic, such as sclerosing orbital pseudotumor, orbital myositis, hypophysitis, and hypertrophic pachymeningitis. New and unusual presentations continue to be described, including a number of vascular manifestations. Substantial progress has been made in elucidating the cell types involved in IgG4-RD, and new pathogenic models are being proposed. Although clinicopathologic correlation remains the cornerstone of diagnosis, ancillary tests such as flow cytometry for circulating plasmablasts and PET–computed tomography have high sensitivity, and certain radiologic features are recognized to be particularly suggestive, such as infraorbital nerve enlargement in IgG4-RD orbitopathy. IgG4-RD often responds to steroids but incomplete responses and relapses are common. Rituximab is emerging as a promising new therapy.

Summary The current review summarizes manifestations of IgG4RD that are of particular relevance to neuro-ophthalmic practice.

aDepartment of Neurology, Massachusetts General Hospital/Harvard Medical School

bDepartment of Neuro-Ophthalmology, Massachusetts Eye & Ear Infirmary, Boston, Massachusetts, USA

Correspondence to Bart K. Chwalisz, MD, Instructor in Neurology, Department of Neurology, Massachusetts General Hospital/Harvard Medical School, 15 Parkman Street, Suite 835, Boston, MA 02114, USA. Tel: +1 617 643 5533; e-mail:

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