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Orbital Wegener granulomatosis without systemic findings
A B Kopstein1, T Kristopaitis, T M Gujrati
1Department of Ophthalmology, Loyola University Medical Center, Maywood, Illinois 60153, USA.
Ophthalmic Plastic and Reconstructive Surgery
|December 10, 1999
Summary
This case report details Wegener granulomatosis (WG) presenting solely as bilateral lacrimal gland masses. This localized orbital form of WG occurred without any systemic disease manifestations.
Area of Science:
- Ophthalmology
- Rheumatology
- Pathology
Background:
- Wegener granulomatosis (WG) is a systemic vasculitis.
- Orbital involvement in WG typically occurs with systemic disease.
Observation:
- A 69-year-old presented with bilateral lacrimal gland masses initially diagnosed as orbital pseudotumor.
- Treatment with corticosteroids and radiation was ineffective, and the disease progressed.
- Unilateral enucleation revealed multifocal vasculitis, necrosis, and granulomas consistent with WG.
Findings:
- Histopathology confirmed Wegener granulomatosis (WG) in the enucleated orbital tissue.
- Elevated antineutrophil cytoplasmic antibody (ANCA) titers supported the WG diagnosis.
- The patient exhibited no extraocular signs of WG and remained systemically disease-free.
Implications:
- This case represents the first reported instance of WG presenting exclusively as bilateral lacrimal gland masses.
- Highlights the possibility of localized orbital WG without systemic involvement.
- Emphasizes the importance of considering WG in refractory orbital inflammatory conditions.