A case of compressive optic neuropathy putatively caused by IgG4-related idiopathic orbital inflammation.

DOI 機関リポジトリ HANDLE
  • HARAGUCHI Ai
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • ANDO Takao
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • UEKI Ikuko
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • HORIE Ichiro
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • IMAIZUMI Misa
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • USA Toshiro
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • YAMASAKI Satoshi
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • ORIGUCHI Tomoki
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences
  • KAWAKAMI Atsushi
    Unit of Translational Medicine, Nagasaki University Graduate School of Biomedical Sciences

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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.

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