Lessons of the month 2: Retinal vasculitis: a first presentation of Takayasu's arteritis

Abstract

Takayasu's arteritis is a chronic, systemic, large-vessel vasculitis affecting the aorta and its primary branches. However, coronary, renal and pulmonary arteries and small vessel involvement has been documented. We describe a rare case of Takayasu's arteritis with extensive supra-aortic arch disease, manifesting with bilateral occlusive retinal vasculitis as a first presentation. This is elicited by fundus findings of vascular sheathing and fundus fluoresceine angiography evidence of retinal vessel occlusion and peripheral capillary non-perfusion.

Keywords: Takayasu's arteritis; retinal vasculitis.

Fig 1.

Bilateral fundus photography and fundus fluorescein angiography at presentation. a) Left fundus showing multiple cotton-wool spots and vascular sheathing in the posterior pole, and microaneurysms and small blot haemorrhages in all four quadrants (arrows). b) Right fundus showing sparse dot retinal haemorrhages in the superotemporal fundus. c and d) Wide-field fundus fluorescein angiography showing bilateral occlusive vasculitis, widespread in the left retina with peripheral capillary non-perfusion, the arrows indicate an occluded retinal vessel (c) and localised in the right temporal and nasal peripheral retina (d).

Fig 2.

Contrast magnetic resonance imaging (MRI) and magnetic resonance angiography of the brain. a) Coronal MRI SENSE sequence demonstrating irregularity of the posterior brain circulation with occluded left V4 branch (arrow). b and c) Axial brain MRI diffusion-weighted imaging / apparent diffusion coefficient sequences showing an acute left precentral gyrus cortical infarct, seen as restricted diffusion (circles).

Fig 3.

Computed tomography angiography of the aortic arch and supra-aortic vasculature. a, b and c) Axial (a), coronal (b) and sagittal (c) images demonstrating occluded left common carotid artery from its origin at the aortic arch (arrows). d) Coronal image showing occluded right external carotid artery (arrow), left common carotid artery (lower dashed arrow) and left internal carotid artery (upper dashed arrow). e) Sagittal reformatted computed tomography demonstrating 90% stenosis of the right internal carotid artery (arrow).

Fig 4.

Bilateral fundus photography showing an improvement in retinal signs 24 weeks from treatment initiation.