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A 33 year old man with left upper lid swelling
Digital Journal of Ophthalmology 2004
Volume 10, Number 12
December 18, 2004
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Lucas Maischner | Kings College Hospital
Kashif Qureshi | St George's Hospital
Fiona Robinson | Kings College Hospital
Scott Robiee | St George's Hospital
Shahram Kashani | Kings College Hospital
Neelanjana Dutt | Kings College Hospital
Diagnosis and Discussion
Wegener’s granulomatosis (WG) is a systemic vasculitis principally affecting the medium and small arteries, venules and arterioles. The condition has a slight male preponderance and a peak incidence in the fourth and fifth decades of life.

WG is classified into three forms. The ‘generalised’ form where systemic vasculitis primarily affects the upper and lower respiratory tracts and kidneys. The ‘limited’ form is confined typically to the respiratory tract and the ‘very limited’ form (which as in our patient) can just affect the orbit. Overall 95% of patients have lung disease, 90% sinus disease and 85% renal disease.[1]

Ocular disease occurs in 50-60% of patients with WG and may be the presenting finding in 13-16%. The most common ocular manifestation is that of orbital involvement (either extending from or independent of sinus involvement).

Presenting symptoms may include awareness of an orbital mass, epiphora, orbital pain and diplopia. Signs may include an orbital mass or proptosis (69%), nasolacrimal duct obstruction (52%), limited ocular rotations (52%), lid erythema and oedema (31%), bony destruction (21%) and reduced visual acuity (17%).[2]

Other frequent findings are conjunctivitis, episcleritis (often preceding scleritis) and peripheral ulcerative keratitis . Less common ophthalmic manifestations include necrotizing scleritis , uveitis , retinitis with venous congestion, optic neuropathy and extra ocular muscle limitation.

Orbital WG may be uni- or bilateral, the latter suggesting disease of greater severity. [3] Our patient had unilateral disease with no sinus involvement which is unusual in relapse of WG.

The main differential diagnosis to consider in this patient was secondary
lymphoproliferative disorder from previous immunosuppressive treatment . However this possibility was unlikely as the histology of the orbital lesion was consistent with a diagnosis of Wegener’s granulomatosis. Although the classic histological triad of vasculitis, tissue necrosis and granulomatous inflammation was not present a review article from the mayo clinic showed that this triad was only present in 54% of orbital WG.[4]

Furthermore the bone marrow trephine and aspirate was normal and a whole body CT failed to demonstrate any evidence of lymphadenopathy .

This is an important distinction to make, as the clinical picture of orbital WG is often similar to that of T-cell lymphomas [5] ( polymorphic reticulosis/lethal midline granuloma) and B-cell lymphomas [6], the treatment of which is radically different.

Nasal and paranasal sinus involvement, resulting in symptoms of progressive nasal obstruction and bloody rhinorrhoea (as opposed to the rapid localized destruction seen in malignant lymphomas) may suggest a diagnosis of WG . The presence of florid lid xanthelasmata has also been reported as being a useful diagnostic pointer in orbital

A definitive diagnosis of orbital WG requires positive serology and biopsy results suggestive of the disease. Serologic testing for cANCA has been shown to be a useful indicator in cases of ocular inflammation where it is difficult to determine the source of inflammation
( a manifestation of wegener’s granulomatosis).[8]

Computed tomography (CT) and magnetic resonance imaging (MRI) have also proved highly useful. Unenhanced, non fat-supressed T1-weighted MRI sequences provide the best contrast between lesions and normal structures with a marked decrease in the T2 signal being characteristic of WG granulomas.[9 ][3 ]

Changes may be accompanied by signs of dural thickening, enhancements of cerebral infarction and signal abnormalities in the brainstem and white matter. Masses are frequently accompanied by infiltrates of the pterygopalatine fossa and destruction of adjacent bone on CT.

Orbital WG, as with systemic WG, responds well to treatment with
immunosuppressive agents (usually prednisolone and cyclophosphamide). Other regimens have been employed in treatment-resistant cases or when side-effects dictate a different approach . These include the use of such drugs as methotrexate[10], azathioprine and trimethoprim-sulfamethoxazole, the latter having been demonstrated to reduce the rate of relapse.[11] More experimental treatments
have included tumour necrosis factor-blocking drugs, such as Infliximab [12] and Etanercept [13] , and antithymocyte globulin.[14] Surgical intervention has been recommended for refractive cases (where orbital exenteration may be required) and in cases where there is a perceived threat to vision (compression of the optic nerve by a granuloma) .[15]

Successful treatment of orbital WG depends on an early diagnosis. A sound understanding of the various manifestations of the condition is therefore essential.

Untreated, mean survival with WG is 5 months. However approximately 90% of patients respond to cyclophosphamide, with about 75% experiencing complete remission.