ATYPICAL WEGENERʼS GRANULOMATOSIS PRESENTING AS LACRIMAL GLAND ENLARGEMENT AND OPHTHALMIC VENOUS OUTFLOW OBSTRUCTION CAUSING SECONDARY GLAUCOMA IN A MAN WITH A PRIOR PAROTIDECTOMY.

Abstract

Purpose of Study Report occurrence of Wegener9s granulomatosis presenting as ophthalmic venous outflow obstruction causing secondary glaucoma in a man with a prior parotidectomy. Methods Used Retrospective case study. Summary of Results A 52-year-old man with diabetes mellitus presented with a red, painful eye. The patient denied dry eyes or dry mouth and otherwise felt well. There was no weight loss, fever, or night sweats. A year earlier, he had had left parotidectomy, which showed nonspecific chronic inflammation. On examination, the intraocular pressure was elevated, and there was episcleral injection, suggesting orbital or ophthalmic venous obstruction causing glaucoma. Magnetic resonance imaging of the brain and orbits showed prominence of the left lacrimal gland and subtle signs of venous congestion bilaterally. Biopsy showed a benign lymphoepithelial lesion with prominent chronic inflammation between acini and ducts, composed of lymphocytes, plasma cells, and eosinophils without granulomata. Later the patient complained of sinus congestion. CT of the sinuses showed septal deviation to the left and enlarged inferior turbinates. There were no intranasal lesions. A Schirmer9s test showed no xerophthalmia, and the oral mucosa was normal. The antiproteinase 3 antibody was > 6 (positive), suggestive of Wegener9s granulomatosis. The antinuclear antibody was weakly positive at 1:40, speckled; the anti-smRNP antibody was 9 (equivocal). The rheumatoid factor was negative. The anti-dsDNA, antimyeloperoxidase, anti-Smith, and SS-A (anti-Ro) and SS-B (anti-La) antibodies were negative; the angiotensin-converting enzyme was within normal limits. Despite therapy with prednisone, the patient developed uveitis and scleritis in both eyes and ultimately responded to therapy with oral cyclophosphamide. The patient was well and chose to stop therapy after 3 years. Conclusions Systemic vasculitis may present with tumor-like lesions.1 Retro-orbital pseudotumors, scleritis, and unilateral parotid enlargement are known Wegener9s manifestations.2,3 The present patient had unilateral parotitis, acute glaucoma secondary to orbital venous outflow obstruction, lacrimal enlargement, and, later, uveitis reflecting regional inflammation. The anti-proteinase 3 antibody suggested Wegener9s granulomatosis. 1. Medicine 2000;79:349-59. 2. Mayo Clin Proc 1990;65:1110-7. 3. Laryngoscope 2004;114:1730-3.