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

  • Oculoplastics and Orbit
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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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Acknowledgments

Funding

CFS Education, New Delhi, and Hyderabad Eye Research Foundation, Hyderabad.

Author contribution

Design and conduct of the study (KM); collection (KM, EA), management (EA, SH), analysis (KM, EA), and interpretation of the data (KM, SH); and preparation, review, or approval of the manuscript (KM, EA, SH).

Conflict of interest

All authors certify that they have NO affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

Financial support:

CFS Education, Centre For Sight, New Delhi, India

Hyderabad Eye Research Foundation, Hyderabad, India

The funding organization had no role in the design or conduct of this research.

Author information

Authors and Affiliations

  1. National Reporting Centre for Ophthalmic Pathology (NRCOP), Centre For Sight, Ashoka Capitol Building, Banjara Hills, Road No.2, Hyderabad, 500034, India

    Kaustubh Mulay

  2. Ophthalmic Pathology Service, L.V.Prasad Eye Institute, Hyderabad, India

    Kaustubh Mulay

  3. Department of Ophthalmic plastics, Vasan Eye Care Hospitals, Hyderabad, India

    Ekta Aggarwal

  4. Ophthalmic and Facial Plastic Surgery and Ocular Oncology, Centre For Sight, Hyderabad, India

    Santosh G. Honavar

  5. Ocular Oncology and Oculoplasty, L.V.Prasad Eye Institute, Hyderabad, India

    Santosh G. Honavar

Authors
  1. Kaustubh Mulay
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  2. Ekta Aggarwal
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  3. Santosh G. Honavar
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Corresponding author

Correspondence to Kaustubh Mulay.

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Mulay, K., Aggarwal, E. & Honavar, S.G. Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review. Graefes Arch Clin Exp Ophthalmol 253, 803–809 (2015). https://doi.org/10.1007/s00417-014-2905-y

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  • DOI: https://doi.org/10.1007/s00417-014-2905-y

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