Wegener’s Granulomatosis: Ophthalmic Manifestations and Management

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Objectives

To provide an up-to-date and comprehensive review of Wegener’s granulomatosis (WG) as a disease entity, focusing on the ophthalmic manifestations and management options.

Methods

A search of Medline was undertaken between 1966 and 2005 regarding WG, systemic vasculitis, and the ocular manifestations of WG. Major ophthalmic and medical textbooks also were reviewed for content, as well as original references.

Results

Involvement of ocular and orbital structures in patients with WG is common and may be a presenting feature. The ocular manifestations range from mild conjunctivitis and episcleritis to more severe inflammation with keratitis, scleritis, uveitis, and retinal vasculitis. Involvement of the nasolacrimal system and orbital tissues also can occur. Except for some cases of anterior segment inflammation, the ocular involvement will not respond to topical agents, but rather to systemic antiinflammatory and immunosuppressive regimens. Surgical intervention may be of value for obtaining tissue diagnosis, in achieving orbital decompression in cases of significant orbital disease with optic nerve compromise, or in cases of nasolacrimal duct obstruction.

Conclusion

WG is an important clinical entity that needs to be recognized early and treated appropriately. Ophthalmic manifestations are frequently encountered and can result in significant morbidity and even blindness. The management is challenging and often requires a multidisciplinary approach.

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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