Lesson of the month 2: A case of Behçet’s disease: 70% have ophthalmic involvement

ABSTRACT

A 34-year-old man presented to the emergency department with acute painless loss of vision of the left eye. Past medical history included painful lumps in the legs and frequent mouth ulcers, which were undiagnosed. The patient’s visual acuity was 6/5 and counting fingers in the right and left eye, respectively. There were extensive intraretinal haemorrhages and venous sheathing in the superior quadrant of the left eye with associated disc oedema. The case was discussed in a multidisciplinary team meeting in the presence of ophthalmology, dermatology and immunology and a diagnosis of Behçet’s disease was reached. The patient was commenced on intravenous methylprednisolone for 3 days followed by a switch to oral prednisolone. Due to recalcitrant uveitis, an intravitreal dexamethasone implant was administered. Eventually, systemic azathioprine and infliximab were commenced with frequent review by ophthalmology and immunology. The macular oedema improved but, unfortunately, the patient’s visual acuity did not recover. Behçet’s disease is a complex vasculitis involving multiple organ systems. Ocular manifestations can occur in 70% of patients, comprising retinal vasculitis, anterior uveitis, iridocyclitis, chorioretinitis, scleritis, keratitis, vitreous haemorrhage, optic neuritis, conjunctivitis, retinal vein occlusion and retinal neovascularisation. A tailored multidisciplinary approach is required, with corticosteroids being the mainstay of treatment.