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Blinding vasculitis: bilateral severe vision loss as a presenting feature of undiagnosed Takayasu arteritis

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© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A middleaged man in his 50s presented with poor vision in both eyes,… Click to show full abstract

© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A middleaged man in his 50s presented with poor vision in both eyes, left eye (OS) more than the right eye (OD) since 1 week. He gave a history of headache, jaw claudication and easy fatigability for the last few weeks. He was a chronic smoker and alcoholic for the past 20 years and denied a history of any significant systemic illness. Bestcorrected visual acuity of OD was counting fingers at 4 m and no light perception in OS. A relative pupillary defect in OS, the anterior segment, was unremarkable in both eyes. Fundus examination revealed scattered cotton wool spots in the posterior pole with patchy retinal whitening in OD (figure 1A) and pallid disc oedema with central retinal artery occlusion (CRAO) in OS (figure 1B). Optical coherence tomography (OCT) confirmed patchy inner and middle layer hyperreflectivity in OD and diffuse inner layer hyperreflectivity in OS. The findings were consistent with the diagnosis of ischaemic retinopathy in OD and combined arteritic anterior ischaemic optic neuropathy (AAION) with CRAO in OS. In view of bilateral ocular ischaemia, he was advised thorough systemic evaluation. Examination revealed absent upper limb pulses with unrecordable blood pressure (BP). In the lower limbs, BP was 140/80 mm Hg. CT angiography of the thorax showed absent flow in the left subclavian artery with normal flow in the aorta and right subclavian artery. Carotid Doppler studies revealed thrombotic plaques with stenosis. Magnetic resonance angiography of the brain revealed an absence of flow across posterior cerebral arteries with ischaemic changes in occipital and frontoparietal lobes. His C reactive protein titres were 77.6 mg/L. Flourescein angiography studies could not be done as the physician ruled out his fitness for the same. A diagnosis of Takayasu arteritis (TAK) was made and the patient was started on systemic steroids. At 8week followup, OD vision improved to 6/36 with the clearing of cotton wool spots (figure 2A). OS showed disc pallor, resolution of retinal oedema and cotton wool spots (figure 2B). OCT of the macula OD showed normal foveal contour and OS revealed thinning of inner retinal layers (figure 2C,D). TAK is a rare chronic granulomatous inflammationcausing intimal fibrosis and vascular narrowing of the aorta, its branches and pulmonary artery. The prevalence of eye morbidities in TAK has been noted to range from 8.1% to 68%. These manifestations are due to poor blood flow across the aorta, carotid and distal arteries, which may present as global or focal ocular ischaemia. Takayasu retinopathy, retinal findings in patients with TAK, may range from subtle retinal dilatation to severe ischaemia of the retina with neovascularisation. Apart from Takayasu retinopathy, retinal artery occlusion, choroidal ischaemia and nonAAION have been described in patients with TAK. The present case represents a severe form of bilateral ischaemic insult secondary to TAK. Our case is unique in that it presented in an elderly man in contrast to a typical young woman, severe bilateral manifestation and total vision loss in one eye. The OS had no light perception owing to combined AAION with CRAO, which is a very rare presentation. Other differentials like Figure 1 Colour fundus of the right eye (A) showing cotton wool spots and patchy retinal whitening over the macula. Left eye fundus (B) shows a chalky white appearing disc with oedema and retinal whitening with a cherryred spot at the centre. Right eye OCT shows segmental inner and middle layer hyperreflectivity (C) and left eye OCT with diffuse inner layer hyperreflectivity (D). OCT, optical coherence tomography. Figure 2 Followup fundus photos of the right eye (A) showing the clearing of whitening and pale disc in the left eye (B). Thinning of inner layers in right (C) and left eye OCT (D). OCT, optical coherence tomography.

Keywords: artery; takayasu; eye; left eye; figure; vision

Journal Title: BMJ Case Reports
Year Published: 2022

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