Immunoglobulin G4-Related Ophthalmic Disease: A Case Report
Thitiwichienlert S, MD¹, Tangpagasit W, MD¹, Sabsanhor S, MD¹, Patarajierapun P, MD¹
Affiliation : ¹ Department of Ophthalmology, Faculty of Medicine, Thammasat University, Pathum Thani, Thailand
The authors reported a rare case with immunoglobulin (Ig) G4-related ophthalmic disease presented with chronic progressive
bilateral complete ophthalmoplegia and blindness from orbital apex syndrome. MRI brain and orbit demonstrated ill-defined
infiltrative lesions at bilateral orbital apexes, bilateral optic canals, and bilateral Meckel’s caves, causing optic nerve compression
and possibly optic neuropathy with generalized leptomeningeal enhancement at dura, cavernous sinus, and parotid gland.
Lumbar puncture revealed few small lymphocytes, rare monocytes, very rare neutrophils with degenerative cells in background,
and negative for malignancy. Serology titers for IgG subclass 4 (IgG4) had resulted in 5.959 grams per deciliter (g/dL). A dural
biopsy revealed aggregate histiocytes with chronic inflammation and focal foreign body type giant cells. Motility improvement
was achieved in the patient after systemic corticosteroids treatment. IgG4 serology should be considered for workup when
patients present with chronic idiopathic orbital inflammation.
Received 4 Feb 2020 | Revised 13 May 2020 | Accepted 15 May 2020
doi.org/10.35755/jmedassocthai.2020.07.11128
Keywords : IgG4-related ophthalmic disease, Ophthalmoplegia, Orbital apex syndrome
