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Andrea Tooley, James Garrity; Clinical Features of IgG4 Related Orbital Disease and Graves' Ophthalmopathy. Invest. Ophthalmol. Vis. Sci. 2017;58(8):5152.
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© ARVO (1962-2015); The Authors (2016-present)
IgG4 related orbital disease (IgG4-RD) is a recently recognized fibro-inflammatory condition characterized by a dense lymphoplasmacytic infiltrate with IgG4 staining plasma cells. IgG4-RD was initially characterized as dacryoadenitis but since then, involvement of the orbital soft tissue, extraocular muscles (EOM) and peripheral nerves have been described. Primary involvement of the EOM can lead to diagnostic confusion with Graves’ ophthalmopathy (GO). We performed a retrospective, observational study of patients with biopsy proven IgG4-RD, who were initially diagnosed with GO, in order to characterize the similarities and differences between Ig4-RD and GO.
We reviewed medical records of 8 patients with biopsy proven IgG4-RD, who were initially diagnosed with GO. We characterized symptoms, thyroid status, radiologic findings, serologic and histologic findings which led to the diagnosis of IgG4-RD.
6 male and 2 female patients were included. Ages ranged from 34-70 years old. Of the 8 patients, 4 had no history of thyroid disease, 3 patients had hyperthyroidism treated with radioactive iodine or methimazole, and 1 patient was on replacement therapy for primary hypothyroidism. Asthma was present in 4/8 patients. The most common presenting symptom was proptosis (7/8 patients). Exophthalmometry ranged from 11-37mm (average 28mm). Orbital decompression had been previously performed in 3/8 patients.Imaging showed enlarged EOM bilaterally in 7/8 patients. The inferior and superior recti were involved in 7/8, and lateral and medial recti in 5/8 patients. All patients had sparing of the muscle tendons. Lacrimal gland enlargement was noted in 2/8 patients. Enlarged infraorbital nerves were seen in 4/8 patients. 3/8 patients had orbital masses. EOM biopsy showed CD20 positive lymphocytes with IgG4 plasma cells in 7/8 specimens. 3/8 patients had elevated serum IgG levels, but only 1 had elevated serum IgG4. 8/8 patients were treated with systemic rituximab and had improvement in their symptoms.
IgG4-RD is a distinct clinical entity and can often be misdiagnosed as GO. GO remains a clinical diagnosis and there is no pathognomonic laboratory or clinical finding. The absence of lid findings and TSH receptor antibodies argue against GO as a diagnosis. Patients may have both GO and IgG4 RD affecting their eyes, therefore, clinicians must be aware of the defining clinical features of each process.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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