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Chung, Lee, You, Cho, Park, and Ahn: A Typical Case of IgG4-related Ophthalmic Disease Satisfying Diagnostic Criteria
Case Report

Journal of the Korean Ophthalmological Society 2017; 58(7): 852-856.

Published online: 14 July 2017

DOI: https://doi.org/10.3341/jkos.2017.58.7.852

A Typical Case of IgG4-related Ophthalmic Disease Satisfying Diagnostic Criteria

Sae Rom Chung, MD1, Tae Eun Lee, MD1, In Cheon You, MD, PhD1, 2, 3, Nam Chun Cho, MD, PhD1, 2, 3, Ho Sung Park, MD, PhD2, 3, 4, Min Ahn, MD, PhD1, 2, 3

1Department of Ophthalmology, Chonbuk National University Medical School, Jeonju, Korea.

2Research Institute of Clinical Medicine, Chonbuk National University, Jeonju, Korea.

3Biomedical Research Institute, Chonbuk National University Hospital, Jeonju, Korea.

4Department of Pathology, Chonbuk National University Medical School, Jeonju, Korea.

Address reprint requests to Min Ahn, MD, PhD. Department of Ophthalmology, Chonbuk National University Hospital, #20 Geonji-ro, Deokjin-gu, Jeonju 54907, Korea. Tel: 82-63-250-1960, Fax: 82-63-250-1960, ahnmin@jbnu.ac.kr

Received 13 October 2016       Revised 6 December 2016       Accepted 17 June 2017

©2017 The Korean Ophthalmological Society

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

In the present study, a case of IgG4-related ophthalmic disease which met the 2015 IgG4-related ophthalmic disease diagnostic criteria is reported and literature review performed.

Case summary

A 51-year-old female presented with both upper eyelid swelling, redness, and a palpable mass. Eye movements were normal and exophthalmos was not observed. Facial computed tomography showed both lacrimal gland hypertrophy. The patient underwent left anterior orbitotomy with incisional biopsy. Immunostained biopsy showed the ratio of IgG4+ to IgG+ cells was 50% and the mean number of IgG4-positive plasma cells was approximately 150 per high-power field. Hematological examination showed elevated serum IgG4 concentrations of 6,930 mg/dL, however, other organs were not involved. The patient satisfied the diagnostic criteria for IgG4-related ophthalmic disease. The patient was given an oral steroid and immunosuppressant and the symptoms improved.

Conclusions

IgG4-related ophthalmic disease is currently receiving increased attention. The authors of the present study report a case of IgG4-related ophthalmic disease which met the 2015 IgG4-related ophthalmic disease diagnostic criteria.

Keywords: Eyelid, IgG4-related disease, IgG4-related ophthalmic disease, Lacrimal gland

Figures and Tables

Figure 1

Photograph and orbital computed tomography of the patient. (A) Both upper eyelid swelling was observed at the first visit. (B) Orbital computed tomography revealed bilaterally enlarged lacrimal glands.

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Figure 2

Biopsied specimens from lacrimal gland. (A) Gross finding of the eyelid mass. (B) Low-power view of the mass showed diffuse lymphoid proliferation consisting of hyperplastic follicles of variable size and intervening sclerotic area (H-E stain, ×20). (C, D) High-power view of the mass showed entrapped epithelial glands in the sclerotic stroma and a lot of plasma cells (arrows) (H-E stain, ×400). (E) There were many plasma cells with IgG immunoreactivity (IgG, ×100, Inset: ×400). The mean number of IgG-positive plasma cells (arrows) was about 300 per a high-power field. (F) There were many plasma cells with IgG4 immunoreactivity (IgG4, ×100, Inset: ×400). The mean number of IgG4-positive plasma cells (arrows) was about 150 per a high-power field.

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Table 1

Diagnostic criteria for IgG4 related ophthalmic disease, 20142

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Diagnosis is classified as “definitive” when (1), (2), and (3) are satisfied; “probable” when (1) and (2) are satisfied; and “possible” when (1) and (3) are satisfied.

Notes

Conflicts of Interest The authors have no conflicts to disclose.

References

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7. Kubota T, Katayama M, Moritani S, Yoshino T. Serologic factors in early relapse of IgG4-related orbital inflammation after steroid treatment. Am J Ophthalmol. 2013; 155:373–379.
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