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Journal of Ophthalmic Inflammation and Infection

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Open Access 01-12-2015 | Brief report

IgG4-associated orbital and ocular inflammation

Authors: Cecilia S Lee, George J Harocopos, Courtney L Kraus, Aaron Y Lee, Gregory P Van Stavern, Steven M Couch, P Kumar Rao

Published in: Journal of Ophthalmic Inflammation and Infection | Issue 1/2015

Abstract

Background

IgG4-associated orbital and ocular inflammation is a relatively unknown entity characterized by sclerosing inflammation with infiltration of IgG4-positive plasma cells. Some so-called idiopathic inflammation syndromes are being re-classified as IgG4-associated inflammation with histopathologic evaluation.

Findings

We report three cases with differing manifestations of IgG4-associated ocular and orbital inflammation: a case of recurrent, treatment-refractory sclero-uveitis that was diagnosed as granulomatosis with polyangiitis with an IgG4-related component, a case of pachymeningitis with optic neuritis that resulted in permanent visual loss, and a case of orbital inflammatory pseudotumor. All three would have been incompletely diagnosed without thorough histopathologic evaluation (including immunohistochemistry).

Conclusions

IgG4-associated disease is an idiopathic, multi-organ inflammatory state that can manifest as chronic, relapsing, sclerosing inflammation in virtually any organ system. There is a wide range of presentations in ocular and orbital inflammation. Ophthalmologists should keep IgG4-associated inflammation in mind when examining chronic, sclerofibrosing inflammation with multi-system involvement. The histology of biopsy specimens is crucial in making the correct diagnosis. Timely assessment may lead to fewer diagnostic tests and more targeted therapy.

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Title: IgG4-associated orbital and ocular inflammation
Authors: Cecilia S Lee
George J Harocopos
Courtney L Kraus
Aaron Y Lee
Gregory P Van Stavern
Steven M Couch
P Kumar Rao
Publication date: 01-12-2015
Publisher: Springer Berlin Heidelberg
Published in: Journal of Ophthalmic Inflammation and Infection / Issue 1/2015
Electronic ISSN: 1869-5760
DOI: https://doi.org/10.1186/s12348-015-0047-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

IgG4-associated orbital and ocular inflammation

Abstract

Background

Findings

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Findings

Conclusions

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