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IgG4-Related Orbital Inflammation

  • Chapter
Orbital Inflammatory Diseases and Their Differential Diagnosis

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

Abstract

IgG4-related inflammation is a recently recognized fibro-inflammatory condition, characterized by tumefactive lesions containing dense lymphoplasmacytic infiltrates rich in IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis that are associated with elevated serum IgG4. Orbital and adnexal tissues are the fifth most common extrapancreatic site. It is mostly seen in middle-aged patients with equal involvement of men and women. IgG4-related inflammation can affect any tissue in the orbit, but the lacrimal gland (88 %) is the most commonly involved, followed by trigeminal nerve branch enlargement (39 %), extraocular enlargement (25 %), and diffuse infiltration of orbital fat (23 %). Patients usually present with a mass or mass-related symptoms and mild or no signs of inflammation. The diagnostic criteria include diffuse/localized masses in single or multiple organs, elevated serum IgG4 concentrations (≥135 mg/dl), and histopathological examination showing marked lymphocytes and plasma cell infiltration and fibrosis, with IgG4+ plasma cells comprising more than 40 % of the total plasma cell population and with at least 10 IgG4+ plasma cells per high-power microscopic field. On magnetic resonance imaging, orbital lesions are hyperintense on T1-weighted and hypointense on T2-weighted images. They enhance with gadolinium and have no destructive bony lesion. IgG4-related inflammation is thought to be a particular Th-2-mediated inflammatory response that may be initiated by particular autoantigens that become recognized during the inflammatory process. There is a delicate balance between Th-2 and Treg cells in the evolving inflammatory responses, and IgG4 behaves as a surrogate marker of a particular type of pro-fibrotic inflammatory process in which it serves to mitigate inflammatory response. IgG4-related inflammation shows a good initial response to corticosteroids, but as the disease evolves into sclerotic phase, it becomes resistant. In these cases, immunosuppressants and rituximab have been used with some success.

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Compliance with Ethical Requirements

Victor M. Elner and Hakan Demirci declare that they have no conflict of interest.

No human or animal studies were carried out for this article.

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  1. Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, 1000 Wall St, Ann Arbor, MI, 48105, USA

    Victor M. Elner MD, PhD & Hakan Demirci MD

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  1. Victor M. Elner MD, PhD
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Correspondence to Victor M. Elner MD, PhD .

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  1. Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, Ann Arbor, Michigan, USA

    Hakan Demirci

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Elner, V.M., Demirci, H. (2015). IgG4-Related Orbital Inflammation. In: Demirci, H. (eds) Orbital Inflammatory Diseases and Their Differential Diagnosis. Essentials in Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46528-8_3

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  • DOI: https://doi.org/10.1007/978-3-662-46528-8_3

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