The posterior segment is rarely involved, but can manifest as retinitis, vasculitis, chorioretinal striae and retinal edema secondary to posterior scleritis. Ocular findings are occasionally the presenting features of the disease. Pathogenesis of Wegener's Granulomatosis is unclear, but is thought to be immune mediated. Diagnosis depends on the clinical presentation, laboratory testing, and pathology. The C-ANCA blood test is believed to be highly sensitive and specific for this disease.
Definitive diagnosis is made by histopathological examination.
Typical findings include necrotizing granulomatous inflamation which may be with or without multi-nucleated giant cells. Treatment relies on the suppression of the immune system with systemic cyclophosphamide and systemic steroid. It is important to note that ocular manifestations of Wegener's need to be treated with systemic immunosuppression. All rights reserved. Reproduction in whole or in part in any form or medium without express written permission of the Digital Journal of Ophthalmology is prohibited.