A Case of Choroidal Folds in a Patient with Immunoglobulin G4-related Ophthalmic Disease

Purpose: To report a case of choroidal folds caused by enlargement of the extraocular muscles (EOM) and orbital fat in a patient with immunoglobulin (Ig) G4-related ophthalmic disease. A full recovery was achieved after steroid administration. Case summary: A 56-year-old male received intravenous high-dose steroids to treat IgG4-related ophthalmic disease presenting with bilateral eyelid edema, hypertrophy of all EOM, and mild exophthalmos. After 3 months, only slight improvement was observed and azathioprine was initiated. The patient exhibited only a minimal response over the next 2 months; all medications were therefore stopped. One month later, the patient presented with aggravation of the swelling (both eyelids), exophthalmos, and visual impairment of the left eye. The corrected vision was 1.0 in the right eye and 0.2 in the left eye; choroid folds were observed in the posterior pole of the left eye. Intravenous steroid (500 mg) was given once and oral steroids prescribed as maintenance therapy. After 2 weeks, the corrected visual acuity recovered to 1.0 in both eyes, the eyelid edema and exophthalmos improved and the choroidal folds resolved completely. Conclusions: Choroidal folds associated with enlargement of EOM and orbital fat tissue can cause visual impairment in patients with IgG4-related ophthalmic disease. Steroids are effective in the acute phases and prevent irreversible visual impairment.