Images in Clinical Medicine

Destructive Eye Lesions in Wegener's Granulomatosis

Peer Malte Aries, M.D., and Marcus Both, M.D.

N Engl J Med 2005; 352:392January 27, 2005

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A 33-year-old woman presented with malaise, bloody nasal discharge, nasal crusting, and arthralgia. On examination, disconjugate gaze and protrusion of the right eye with scleritis were found. Serologic evaluation revealed elevated acute-phase reactants. Antineutrophil cytoplasmic antibodies (ANCA) with a cytoplasmic pattern, at a titer of 1:256, were positive on direct immunofluorescence staining, and the level of ANCA directed against proteinase 3 was more than 100 U per milliliter in an enzyme-linked immunosorbent assay (normal value, <5). Examination of a sinus-biopsy specimen revealed granulomatous inflammation with geographic necrosis, and Wegener's granulomatosis was diagnosed. Therapy with cyclophosphamide and prednisone was initiated. Increasing retro-orbital pain, necrotizing inflammation of the right lower eyelid, collapse of the nasal bridge, and a cutaneous defect of the medial eyelid with fistulization to the pharynx developed (Panel A). A computed tomographic scan showed a retro-orbital mass and destruction of the orbital wall (Panel B). Intensified immunosuppressive therapy was administered, with a good outcome. One year later, the patient's disease is under control with a maintenance regimen of immunosuppressive therapy with azathioprine.

Peer Malte Aries, M.D.
Rheumaklinik Bad Bramstedt, 24576 Bad Bramstedt, Germany

Marcus Both, M.D.
University Hospital Schleswig-Holstein, D-24105 Kiel, Germany

Citing Articles (4)

Citing Articles

1. 1

   Stephan D. Gadola, Wolfgang L. Gross. (2012) Vasculitis in 2011: The renaissance of granulomatous inflammation in AAV. Nature Reviews Rheumatology
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2. 2

   Simon R J Taylor, Alan D Salama, Charles D Pusey, Susan Lightman. (2007) Ocular manifestations of Wegener’s granulomatosis. Expert Review of Ophthalmology 2:1, 91-103
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3. 3

   W. A. Schmidt, M. Both, E. Reinhold-Keller. (2006) Bildgebende Verfahren in der Rheumatologie: Bildgebung bei Vaskulitiden. Zeitschrift für Rheumatologie 65:7, 652-661
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4. 4

   Wolfgang A Schmidt, Annette D Wagner. (2006) Role of imaging in diagnosis of and differentiation between vasculitides. Future Rheumatology 1:5, 627-634
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