THE CLINICAL AND PATHOLOGICAL CONSTELLATION OF WEGENER GRANULOMATOSISOF THE ORBIT

Purpose: Wegener granulomatosis (WG) may present as an orbital mass wi thout obvious upper respiratory or systemic features, The authors exam ined the clinical and pathologic features of a series of cases of orbi tal 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 i ndex of diseases. Clinical features were correlated with the results o f computed tomography in 12 cases and orbital biopsy in 11 cases, Anti neutrophil cytoplasmic antibody (c-ANCA) testing was performed in five cases. Results: The main ocular symptoms were decreased vision, redne ss, and ocular and facial pain, whereas the main signs were proptosis, scleritis, and lid inflammation. Progression was marked by an increas ed incidence of bilaterality and systemic features. Ear, nose, and thr oat features were discovered at presentation in 11 cases and became un iversal during the follow-up period. Initial antineutrophil cytoplasmi c antibody test results were negative in five patients but became posi tive later in three patients. Orbital biopsy specimens typically had f eatures of mixed inflammation, fat disruption, and small areas of necr osis. The combination of cyclophosphamide and oral steroids was highly effective in terminating disease episodes, Conclusions: Orbital WG ca n be recognized by a constellation of clinical and radiologic findings with evidence of an often erosive, infiltrating, and restrictive fibr otic, inflammatory mass. Concurrent ear, nose, and throat or specific ocular findings such as scleritis with typical limbal infiltrate can o ccur. Biopsy results show mixed inflammation with evidence of necrosis that must not be regarded as a nonspecific finding.