Neuro-ophthalmic complications of IgG4-related disease.

Curr Opin Ophthalmol

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

Published: November 2018

AI Article Synopsis

  • * Recent findings show that IgG4-RD is becoming recognized as a common cause of various previously unexplained disorders in the head and neck, including conditions affecting the eyes and brain; new presentations and better understanding of the underlying biology are also emerging.
  • * Diagnosis primarily relies on the correlation between clinical features and pathology, with advanced tests like flow cytometry and PET-CT improving detection; treatment typically involves steroids, but new

Article Abstract

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.

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Source
http://dx.doi.org/10.1097/ICU.0000000000000523DOI Listing

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