Elevated anti-neutrophil cytoplasmic antibody titer in a patient with atypical orbital pseudotumor.

We recently examined a patient with ophthalmic manifestations of Wegener's granulomatosis in whom antineutrophil cytoplasmic antibody (cANCA) titers provided helpful diagnostic information. A 40-year-old man who had suffered from bilateral exophthalmos for 9 months was diagnosed initially as having idiopathic inflammatory pseudotumor. The patient exhibited purulent nasal discharge and microhematuria. A histopathologic study revealed vasculitis. His ANCA titer for cANCA was found to be evaluated, and our patient was subsequently diagnosed as having Wegener's granulomatosis. His ocular symptoms resolved and did not recur after treatment with corticosteroid in combination with cyclophosphamide. We believe that cANCA levels should be investigated in patients with orbital pseudotumor as a possible sign of Wegener's granulomatosis.