Background: In IgG4-related ophthalmic disease (IgG4-ROD), visual acuity and visual field are maintained in many cases, while the lacrimal glands, trigeminal nerves and extraocular muscle are enlarged. In some cases, however, visual loss and/or visual field defect occur; such a condition is referred to as IgG4-related optic neuropathy. Here we report two cases of optic neuropathy in two patients with extremely high IgG4 levels (≥ 1,000 mg/dL) at the initial examination.
Case 1: A 52-year-old man presented with impaired visual acuity and visual field in the right eye. As elevated serum levels of IgG4 (1,380 mg/dL) and enlargement of the extraocular muscles and trigeminal nerve were observed, he was diagnosed with "possible" IgG4-ROD. Steroids were administered orally in a tapered manner, starting at 80 mg/day of prednisolone (PSL). He is currently receiving maintenance therapy with 8 mg/day of PSL. Although the treatment resulted in improvement of the enlargement of the extraocular muscles and trigeminal nerve in both eyes, irreversible central scotoma remained in the right eye.
Case 2: A 43-year-old man presented with a visual field abnormality in the right eye. As elevated serum levels of IgG4 (1,440 mg/dL), enlargement of the lacrimal gland and trigeminal nerve, as well as a tumor-like mass in the orbital apex were noted, he was diagnosed with"possible"IgG4-ROD. Steroids were administered orally in a tapered manner, starting at 40 mg/day of PSL. He is currently undergoing maintenance therapy with 8 mg/day of PSL. Following treatment, the tumor-like mass in the orbital apex decreased in size, and the right visual field defect also improved.
Conclusions: Cases of IgG4-ROD exhibiting extremely high serum IgG4 levels (≥ 1,000 mg/dL) may develop optic neuropathy and suffer from irreversible visual acuity and visual field defects even after treatment. Therefore, early diagnosis and treatment are required.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 124: 655-665, 2020.