A Case of Orbital Involvement in IgG4-Related Disease

A 46-year-old male was referred to the Ophthalmology Service for a 7-year history of bilateral proptosis and a presumptive diagnosis of thyroid eye disease. Past medical history was only significant for autoimmune pancreatitis. All laboratory testing including tests of thyroid function were within normal limits. The patient underwent orbital biopsy and was found to have plasma cells containing mainly IgG4 immunoglobulin that was consistent with IgG4-related disease. The patient was treated with oral prednisone and the proptosis resolved within 3 weeks.     

A Clinicopathologic Case Study of Two Patients with Pediatric Orbital IgG4-related Disease

Abstract

The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.     

The Case of IgG4-Related Ophthalmic Disease with Perivascular Lesions of Superior Ophthalmic Vein Associated with Optic Nerve Disturbance

A 61-year-old man with hyperthyroidism had exophthalmos with dilated conjunctival vessels in both eyes. Magnetic resonance imaging showed that the superior ophthalmic veins had a funicular-like appearance. Cerebral angiography showed no blood flow from both internal carotid arteries to the cavernous sinus, thus excluding a carotid-cavernous fistula. Blood tests showed an elevation of IgG4 (281 mg/dl), and a IgG4-related ophthalmic disease was considered. Steroid pulse therapy was performed, and all of the abnormal findings were improved. We concluded that this was a rare case of IgG4-related ophthalmic disease with perivascular lesions of the superior ophthalmic vein associated with optic nerve disturbance.