Orbital Involvement and Ocular Surface Changes in IgG4-Related Systemic Disease

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To evaluate ophthalmic involvement of systemic immunoglobulin-G4–related disease (IgG4-RD) and describe the changes in both ocular surface parameters and corneal subbasal nerve plexus.

Materials and Methods:

Twenty eyes of 10 patients with systemic IgG4-RD and 20 eyes of 10 control subjects were enrolled in this study. Demographic information, medical history, serum IgG4 levels, orbital imaging, and follow-up data of subjects were recorded. Ocular surface tests were carried out in the order of tear break-up time (BUT), lissamine green (LG) staining, Schirmer I test with anesthesia, and ocular surface disease index (OSDI) questionnaire for all participants. Corneal subbasal nerves and basal epithelial cell layer were evaluated using in vivo confocal microscopy.


Among the 10 patients with IgG4-RD, 11 eyes of 7 patients had orbital involvement. Among these 7 patients with IgG4-related ophthalmic disease, 4 presented with painless eyelid or periorbital swelling, 2 with diplopia and restricted ocular motility, and 1 with proptosis. Patients with IgG4-RD had higher OSDI (5.9 ± 6.6 vs. 1.7 ± 2.4, P < 0.001) and LG staining scores (0.7 ± 1.0 vs. 0.0 ± 0.0, P = 0.011) and lower BUT (5.6 ± 1.4 vs. 10.2 ± 1.0, P < 0.001) and Schirmer values (11.9 ± 10.3 vs. 18.3 ± 4.4, P = 0.021) as compared with those of control subjects. Total nerve density and nerve fiber length were found to be significantly lower in patients with IgG4-RD.


The orbit is frequently involved during the course of IgG4-RD. These patients should be evaluated in terms of ocular surface disease and dry eye, which may be associated with lacrimal gland and/or orbital nerve involvement.

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