The clinical and pathologic constellation of Wegener granulomatosis of the orbit.

PURPOSE: Wegener granulomatosis (WG) may present as an orbital mass without obvious upper respiratory or systemic features. The authors examined the clinical and pathologic features of a series of cases of orbital WG to define the features of presentation and progression of this disorder.
METHODS: Thirteen subjects with orbital presentations of WG were identified from the University of British Columbia Orbit Clinic index of diseases. Clinical features were correlated with the results of computed tomography in 12 cases and orbital biopsy in 11 cases. Antineutrophil cytoplasmic antibody (c-ANCA) testing was performed in five cases.
RESULTS: The main ocular symptoms were decreased vision, redness, and ocular and facial pain, whereas the main signs were proptosis, scleritis, and lid inflammation. Progression was marked by an increased incidence of bilaterality and systemic features. Ear, nose, and throat features were discovered at presentation in 11 cases and became universal during the follow-up period. Initial antineutrophil cytoplasmic antibody test results were negative in five patients but became positive later in three patients. Orbital biopsy specimens typically had features of mixed inflammation, fat disruption, and small areas of necrosis. The combination of cyclophosphamide and oral steroids was highly effective in terminating disease episodes.
CONCLUSIONS: Orbital WG can be recognized by a constellation of clinical and radiologic findings with evidence of an often erosive, infiltrating, and restrictive fibrotic, inflammatory mass. Concurrent ear, nose, and throat or specific ocular findings such as scleritis with typical limbal infiltrate can occur. Biopsy results show mixed inflammation with evidence of necrosis that must not be regarded as a nonspecific finding.