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• W2892212957 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." @default.
• W2892212957 created "2018-09-27" @default.
• W2892212957 creator A5028305623 @default.
• W2892212957 creator A5042482253 @default.
• W2892212957 date "2018-11-01" @default.
• W2892212957 modified "2023-10-17" @default.
• W2892212957 title "Neuro-ophthalmic complications of IgG4-related disease" @default.
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• W2892212957 doi "https://doi.org/10.1097/icu.0000000000000523" @default.
• W2892212957 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30199519" @default.
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