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The patient €™s eye at presentation

A 20 year old man was referred to the eye clinic by his general practitioner with a four week history of a sore red left eye (fig 1). Treatment for conjunctivitis had not resulted in improvement, and he reported that his eye was increasingly painful. He had experienced repeated nose bleeds over the past three months, together with constant left sided nasal stuffiness. He had recently felt generally unwell and had lost 4 kg. On examination, his visual acuity was 6/9 in both eyes. His vital signs were stable, but urinalysis showed blood+++ and protein+.

Questions

1 What abnormalities are seen in the photograph?

2 What is the most likely underlying diagnosis given his extraocular symptoms?

3 What further investigations are appropriate?

4 What is the pathophysiology of this condition?

5 What treatment would you recommend and what is the prognosis?

Answers

1 What abnormalities are seen in the photograph?

Long answer

The abnormality is in the sclera €”the tough white outer coat of the globe. A large area of necrotic sclera can be seen extending around the superonasal side of the cornea. Non-necrotising scleritis presents as a red sore eye, but the white avascular area of sclera seen here is indicative of necrotising disease.

2 What is the most likely underlying diagnosis given his extraocular symptoms?

Long answer

Necrotising scleritis is associated with a medical disorder in 39-50% of cases, particularly connective tissue disease or systemic vasculitis. History taking, examination, and investigations should centre on a differential diagnosis of systemic lupus erythematosus; rheumatoid arthritis; Crohn €™s disease; or the antineutrophil cytoplasmic antibody (ANCA) associated vasculitides, including Wegener €™s granulomatosis. Wegener €™s granulomatosis is the most common cause, and the combination of necrotising scleritis, upper airway symptoms, and constitutional symptoms strongly suggests this as the diagnosis.

3 What further investigations are appropriate?

Short answer

The diagnosis of necrotising scleritis is clinical, but if Wegener €™s granulomatosis is suspected, serious renal involvement must be urgently excluded by urinalysis with a dipstick. Radiographic assessment of the respiratory system and formal evaluation for renal disease are indicated. Antineutrophil cytoplasmic antibodies (ANCAs) are usually positive in systemic Wegener €™s granulomatosis but can be negative, particularly if the disease is limited to the orbit.

4 What is the pathophysiology of this condition?

Short answer

Wegener €™s granulomatosis is an autoimmune systemic vasculitis, which commonly affects small and medium sized vessels. The precise causes are not known, and it belongs to a larger group of vasculitides in which disease is strongly associated with ANCAs, including Churg-Strauss syndrome and microscopic polyangiitis.

5 What treatment would you recommend and what is the prognosis?

Short answer

Wegener €™s granulomatosis is life threatening and requires aggressive immunosuppression; cyclophosphamide has revolutionised the management of this previously fatal condition. Once the diagnosis is confirmed, start patients on intravenous infusions of cyclophosphamide and high dose oral prednisolone (1-2 mg/kg/day). Treatment of the underlying condition leads to rapid resolution of necrotising scleritis without the need for local treatment, unless the globe has already perforated, in which case emergency surgery may be needed. Most patients respond well to treatment, and survival is about 95% at five years and 80% at 10 years.

Patient outcome

Our patient was diagnosed with Wegener €™s granulomatosis. Pulmonary nodules were seen on chest radiography and he was positive for ANCAs on direct immunofluorescence and for antiproteinase 3 antibodies on ELISA.

He was started on 80 mg oral prednisolone daily; concurrent infusions of cyclophosphamide were arranged in conjunction with the renal physicians through our multidisciplinary vasculitis clinic, which includes renal doctors; ophthalmologists; and ear, nose, and throat surgeons.

His symptoms improved rapidly with treatment. His nose bleeds and nasal congestion improved, and he rapidly regained some weight and felt better systemically. The necrotising scleritis resolved without compromise to his vision (fig 2).

Fig 2 The patient €™s eye six months later

© 2012 BMJ Publishing Group Ltd

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