Journal List > J Korean Ophthalmol Soc > v.58(7) > 1010817

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

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.

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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