IgG4-positive sclerosing orbital inflammation involving the conjunctiva: a case report.

PURPOSE: To describe IgG4-positive sclerosing orbital inflammation with prominent conjunctival and scleral involvement.

DESIGN

Case report.

METHODS

Clinical, radiologic, and pathologic correlation.

RESULTS

A 66-year-old man presented with right eye redness and irritation. Examination revealed unilateral scleritis and nongranulomatous anterior uveitis with elevated p-ANCA and CRP. Orbital CT scan showed inferotemporal scleral thickening. Biopsy revealed sclerosis and IgG4-positive plasma cells in the conjunctiva and inferior rectus.

CONCLUSIONS

IgG4-mediated sclerosing inflammation is well-recognized in the orbit and adnexa, particularly the lacrimal gland. Scleritis with anterior uveitis should be recognized as a possible presentation for this entity, which has important systemic associations.