Wegener’s Granulomatosis: Ophthalmic Manifestations and Management
Section snippets
Methods
We reviewed the relevant medical literature by searching “Medline” (1966 and 2005), using the combinations of the following keywords: “Wegener’s granulomatosis,” “orbit,” “ocular,” “eye,” and “systemic vasculitis.” Our search was limited to human studies and preference was given to English-language journals. Articles were included if they emanated from peer-reviewed journals. From these studies and their references, 1276 abstracts were reviewed and those pertinent to our discussion were
Results
WG is defined as a small-vessel idiopathic primary vasculitis and, together with Churg-Strauss syndrome (CSS), microscopic polyangiitis and necrotizing pauci-immune glomerulonephritis, demonstrates a strong association with antineutrophil cytoplasmic antibodies (ANCA) (19, 20). Traditionally the diagnostic pathological triad in WG is described as that of parenchymal necrosis, vasculitis, and granulomatous inflammation (21). It is suggested that the demonstration of the classic triad varies
Discussion
WG is a rare entity that can affect almost any organ system. It can often present with nonspecific symptoms and signs, and hence, may be misdiagnosed. Ophthalmic involvement is relatively common in the course of the disease and may in fact be the initial presenting feature in some patients. It is therefore imperative to consider WG in the differential diagnosis, when more common disorders are ruled out. Even if not initially confirmed, it may be necessary to follow patients on a regular basis
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- ⁎
Resident of Ophthalmology.
- †
Professor of Ophthalmology, Chairman.
- ‡
Fellow in Oculoplastic and Orbital Surgery.