Orbital Wegener granulomatosis without systemic findings.

Purpose: To describe a case of orbital Wegener granulomatosis without systemic disease.
Method: Case report.
Results: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with Wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically.
Conclusion: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.