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Graefe's Archive for Clinical and Experimental Ophthalmology
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology 5/2015

01-05-2015 | Oculoplastics and Orbit

Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review

Authors: Kaustubh Mulay, Ekta Aggarwal, Santosh G. Honavar

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 5/2015

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Abstract

Background

Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD.

Material/Methods

This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD.

Results

Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment.

Conclusion

Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.
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Metadata
Title
Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review
Authors
Kaustubh Mulay
Ekta Aggarwal
Santosh G. Honavar
Publication date
01-05-2015
Publisher
Springer Berlin Heidelberg
Published in
Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 5/2015
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI
https://doi.org/10.1007/s00417-014-2905-y

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