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IgG4-Related Ophthalmic Disease

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Ocular Adnexal Lesions

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Abstract

IgG4-related disease (RD) was originally recognized in 2001 in a patient with autoimmune pancreatitis with elevated serum IgG4. Subsequently, a fibro-inflammatory condition, characterized by tumefactive lesions at multiple sites, with dense lymphoplasmacytic infiltrate, rich in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis was found to be associated with autoimmune pancreatitis. The sites include the biliary tree, salivary glands, periorbital tissue, kidneys, lungs, lymph nodes, meninges, aorta, breast, prostate, thyroid, pericardium, and skin. Mikulicz syndrome with lacrimal and salivary gland enlargements is now considered as IgG4-RD. Clinically, the lacrimal gland is the most affected among the ophthalmic tissues, but the others include extraocular muscles, trigeminal nerve, orbital fat, eyelids, and nasolacrimal system. For ophthalmic disease, the term IgG4-related ophthalmic disease (IgG4-ROD) is used.

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Further Reading

  1. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.

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  5. Toyoda K, Oba H, Kutomi K, et al. MR imaging of IgG4-related disease in the head and neck and brain. Am J Neuroradiol. 2012;33(11):2136–9.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology, National University Hospital, Singapore, Singapore

    Shantha Amrith, Stephanie Ming Young & Gangadhara Sundar

  2. Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore

    Shantha Amrith, Stephanie Ming Young & Gangadhara Sundar

  3. Department of Diagnostic Imaging, National University Hospital, Singapore, Singapore

    Eric Ting

  4. Department of Diagnostic Imaging, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore

    Eric Ting

  5. Advanced Medicine Imaging, Singapore, Singapore

    Eric Ting

  6. Department of Pathology, National University Hospital, Singapore, Singapore

    Bingcheng Wu & Min En Nga

  7. Department of Pathology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore

    Min En Nga

Authors
  1. Shantha Amrith
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  2. Stephanie Ming Young
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  3. Eric Ting
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  4. Bingcheng Wu
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  5. Min En Nga
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  6. Gangadhara Sundar
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Corresponding author

Correspondence to Shantha Amrith .

Editor information

Editors and Affiliations

  1. National University Hospital, National University of Singapore, Singapore, Singapore

    Shantha Amrith

  2. National University Hospital, National University of Singapore, Singapore, Singapore

    Gangadhara Sundar

  3. National University Hospital, National University of Singapore, Singapore, Singapore

    Stephanie Ming Young

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© 2019 Springer Nature Singapore Pte Ltd.

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Amrith, S., Young, S.M., Ting, E., Wu, B., Nga, M.E., Sundar, G. (2019). IgG4-Related Ophthalmic Disease. In: Amrith, S., Sundar, G., Young, S. (eds) Ocular Adnexal Lesions. Springer, Singapore. https://doi.org/10.1007/978-981-13-3798-7_11

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  • DOI: https://doi.org/10.1007/978-981-13-3798-7_11

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-3797-0

  • Online ISBN: 978-981-13-3798-7

  • eBook Packages: MedicineMedicine (R0)

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