Unilateral proptosis in a 74-year-old woman

Multisystem disorders can present with protean manifestations. We report a particularly unusual case of a systemic vasculitis in which the principal clinical manifestation was unilateral proptosis caused by an orbital pseudotumour. A 74-year-old woman initially developed nocturnal cough, wheeze, nasal congestion and conjunctival irritation. She was treated with bronchodilators and intranasal steroids without relief. The patient presented four months later with diplopia and left-sided proptosis. An MRI of the orbits revealed increased retro-orbital tissue. Lacrimal gland biopsy showed a polyclonal lymphocytic infiltrate. A presumptive diagnosis of orbital pseudotumour was made and the patient was treated with orbital radiotherapy. This failed to control her worsening proptosis. Increasing headache, malaise, exertional dyspnoea and a 201b weight loss soon followed. Eye examination revealed persistent left proptosis and diplopia on left lateral gaze. The remainder of her physical examination was normal. Haemoglobin was 9.9g/dL, WBC 5.6 (with a normal differential), platelets 461 and ESR 126. Chest X-Ray demonstrated bilateral reticulonodular infiltrates. Serologic studies included a negative ANA and c-ANCA with a weakly,positive p-ANCA and positive antimyeloperoxidase antibodies in a titre of 1:80. A diagnostic video-assisted thoracoscopic lung biopsy was subsequently performed. Histology revealed patchy organizing pneumonia with necrotizing granulomas and vasculitis forming multiple nodules, consistent with Wegener's granulomatosis. The patient was started on 60mg of prednisone daily as well as cyclophosphamide 750mg monthly (for a total of nine doses) and co-trimoxazole bid. The proptosis resolved within days, as did the patient's symptoms. A chest X-ray two months later was completely normal.