IgG4 disease is increasingly recognized as a cause of orbital disease. The authors reviewed the records of 42 patients with biopsy proven IgG4 disease involving the orbit. Patients presented with eyelid swelling (80.9%), proptosis (38.1%), and diplopia (11.9%). The most common location of orbital disease in these patients was the lacrimal gland (83.3%) followed by extraocular muscles (31.1%), trigeminal nerve (23,8%), orbital fat (33.3%), nasolacrimal duct (2.4%), and the eyelid (2.4%).
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