We report the case of a 58-year-old male presenting with an impairment of the left-sided visual acuity caused by compressive
optic neuropathy, and marked bilateral proptosis. Blood test showed markedly elevated IgG4 (1830 mg/dl) and positive
TSH receptor-stimulating antibodies (200%), but the thyroid function test were normal. Orbital MRI revealed abnormal soft tissue
proliferation around the optic nerve and fusiform enlargement of the extraocular muscles. Systemic CT analysis detected
multiple lymph node swelling, pseudotumor in the lung, retroperitoneal fibrosis, and kidney lesions. We considered that the eye
manifestation was most likely caused by IgG4-related idiopathic orbital inflammation. Systemic administration of a moderate
dose of prednisolone dramatically improved the compression of the optic nerve, as shown by the improvement of the visual
acuity and the MRI findings. The clinical course made thyroid-associated ophthalmopathy unlikely. In conclusion, an overall
consideration of the clinical picture and extensive work-up of any possible differential diagnosis including measurement of the
serum levels of IgG4 was highly useful in making the diagnosis of the patient.
A case of compressive optic neuropathy putatively caused by IgG4-related idiopathic orbital inflammation.
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