J Korean Ophthalmol Soc.  2012 Dec;53(12):1879-1884. 10.3341/jkos.2012.53.12.1879.

A Case of IgG4-Related Sclerosing Disease Involving the Optic Nerve

• Cho HS
• Choi JY
• Yum JH

• KMID: 2216405
• DOI: http://doi.org/10.3341/jkos.2012.53.12.1879

Abstract

PURPOSE
To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a.
CASE SUMMARY
A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland's posterior side and the lateral rectus muscle's lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.

Keyword

IgG4-related sclerosing disease; Mikulicz disease; Orbital inflammatory disease

MeSH Terms

Biopsy
Dacryocystitis
Exophthalmos
Eye
Humans
Immunochemistry
Immunoglobulin G
Lymphoma
Magnetic Resonance Imaging
Middle Aged
Mikulicz' Disease
Muscles
Optic Nerve
Steroids
Visual Acuity
Immunoglobulin G
Steroids

Figure

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  Figure 1 Humphrey visual field test reveals marked visual field defects in the left eye (A), and inferior altitudinal visual field defects in the right eye (B).

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  Figure 2 Orbit MRI reveals diffuse well enhancing infiltrative lesions involving both optic nerves, extraocular muscles, and cavernous sinus (A). Diffuse heterogeneous lesions involving the right maxillary sinus suggests sinusitis (B).

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  Figure 3 (A) Biopsied specimen shows interstitial fibrosis and lymphoplasmacytic infiltrations with lymphoid follicles (H&E, ×400). (B) Immunochemistry staining for IgG4 expression: IgG4-positive plasma cells have infiltrated the lesion (Anti-IgG4 Ab, ×400).

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  Figure 4 Two months after steroid therapy, there is significant improvement in the visual field defects of both eyes (A, B).

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  Figure 5 Orbit CT images reveals interval decreased extents of infiltrative lesions involving both optic nerves and extraocular muscles after steroid therapy (A, B).

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