A sore red eye with systemic involvement

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Zaid Shalchi, ophthalmology specialty registrar year 2 and MSc student13,
Susan L Lightman, professor of clinical ophthalmology and consultant ophthalmologist23,
Charles D Pusey, professor of medicine and head of renal medicine24,
Simon R J Taylor, National Institute for Health Research clinical lecturer in ophthalmology123

¹Royal Surrey County Hospital NHS Foundation Trust, Guildford, UK
²Multidisciplinary Vasculitis Clinic, Imperial College Healthcare NHS Trust, Hammersmith Hospital, London, UK
³UCL Institute of Ophthalmology, London, UK
⁴Faculty of Medicine, Imperial College London, Hammersmith Hospital

Correspondence to: S R J Taylor s.r.taylor{at}ucl.ac.uk

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+.

Fig 1 The patient’s eye at presentation

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?

Short 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.

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?

Short answer

Necrotising scleritis can be associated with several connective tissue disorders, the most common being Wegener’s granulomatosis. The combination of necrotising scleritis, upper airway symptoms, and constitutional symptoms strongly suggest this as the diagnosis.

Long answer

Necrotising scleritis is associated with a medical disorder in 39-50% of cases, particularly connective tissue disease or systemic vasculitis.1 2 History taking, examination, and investigations should centre on a differential diagnosis of systemic lupus erythematosus; rheumatoid …