Orbital wegener granulomatosis without systemic findings

Purpose: To describe a case of orbital Wegener granulomatosis without syste mic disease. Method: Case report. Results: A 69-year-old patient with bilateral inflammatory lacrimal gland m asses underwent multiple biopsies that showed a nonspecific lymphoplasmacyt ic infiltrate consistent with orbital pseudotumor. After unsuccessful treat ment with systemic corticosteroids and radiation, severe orbital disease ra pidly progressed and the patient underwent unilateral enucleation. The enuc leated specimen showed multifocal vasculitis, tissue necrosis, and granulom as consistent with Wegener granulomatosis (WG). Elevated antineutrophil cyt oplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemic ally. Conclusion: The authors believe this is the first report of bilateral lacri mal gland masses presenting as a localized form of WG in the total absence of systemic disease.