Takayasu arteritis (TA) is an obstructive arteritic disorder affecting mainly medium to large arteries. It has a relatively high incidence in the Asian population, although cases have been reported from all over the world. It affects the aorta and/or its major branches giving rise to end organ hypoperfusion. Ischaemic symptoms are the hallmark of the disease. The major complications attributed to the disease include Takayasu’s retinopathy, secondary hypertension and arterial aneurysms. It mostly affects young women. The ophthalmic complications generally manifest late in the course of the disease. Anterior ischaemic optic neuropathy (AION) is a rare association with Takayasu disease.
A 30-year-old male presented with a history of repeated attacks of blurring of vision and black curtain over his right eye. The first episode occurred 10 months before presenting to us. Each episode reportedly used to persist for 2-5 minutes followed by a return to normal vision. Six months earlier he had experienced sudden loss of vision in the right eye, and did not recover thereafter. At the same time he had similar intermittent episodes of blurring of vision in his left eye. These episodes were not associated with any loss of colour vision, distortion of objects or floaters. He gave a history of giddiness and syncopal attack, particularly when he extended his neck. Shaking of all four limbs, difficulty in walking and partial loss of consciousness accompanied the attacks. These attacks lasted 1-2 minutes and occurred 4-5 times a day. He denied any history of headache, diplopia, vomiting, arthralgia, fever, sore throat, weight loss or ache. There was no history of hypertension and chest pain. He was a non-smoker and a non-alcoholic.
On initial examination, vitals were stable except for bilateral absence of radial, brachial and carotid pulses. His visual acuity in right eye was counting fingers at half meter with inaccurate projection and 6/36 in the left eye. Anterior segment was normal except for afferent pupillary defect in both eyes. Intraocular pressure (IOP) was 6 mmHg and 12 mmHg in the right and left eye respectively. The media were clear. Fundus showed optic atrophy in right eye [Figure:1] and disc pallor with oedema, dilatation of vessels, and segmentation of blood column in the left eye [Figure:2] and microaneurysms in equatorial region in both eyes. Visual fields could not be done. Fundus fluorescein angiography showed increased arm to choroid time (25.5 seconds), increased AV transit time (41.2 seconds), patchy filling of choroid, microaneurysms [Figure:3], segmentation of blood column and hyperfluorescence of left disc [Figure:4]. Ophthalmodynamometry in right eye was unrecordable and IOP was 20mm of Hg in the left eye. These fundus findings were suggestive of bilateral AION.
Based on history of amaurosis, AION, and obstructive retinal arterial disease, provisional diagnosis of Takayasu arteritis was made and the patient was referred to a cardiothoracic surgeon. The blood pressure (BP) was unrecordable in the upper limbs and lower limb systolic BP recorded by palpating dorsalis pedis artery was 140 mmHg. Neurological, chest and abdominal examination were normal. CT Scan (head) was normal. Laboratory data showed 40mm ESR at first hour. Additional laboratory investigations were done to exclude other causes of arteritis such as syphilis, hepatitis, systemic lupus erythromatosis and Wagene’s granulomatosis. Aortography revealed total occlusion of all the four arch vessels. Few collaterals could be seen emerging from arch of aorta and going to various parts of brain [Figure:5]. Abdominal aorta and renal arteries were spared.
According to ’The American College of Rheumatology 1990, this patient fitted the diagnosis of TA, despite male gender and presenting with AION. The patient was put on Tab. prednisolone 60mg daily and Tab.acetylsalicylic acid 100mg daily for 4 weeks. The ESR recorded 7mm at first hour and there was improvement in his syncopal attacks 4 weeks later. He was advised by the cardiotheraic surgeon to undergo bypass graft.
Takayasu arteritis is characterised by diverse clinical presentation. The development of neurological symptoms such as dizziness, syncope and transient ischaemic attacks depend upon the extent of involvement of cerebral branches of the aortic arch. Visual disturbances like amaurosis, retinal ischaemia and low IOP result from occlusion of central retinal artery and posterior ciliary arteries. Ocular changes have been reported to occur in 60% of the patients of Takayasu disease. The incidence of Takayasu retinitis is reported up to 3.6% in India, USA, and Mexico, and over 25% in Japan. It is known to occur more in females - the female: male ratio varies from 9.4:1 to 1.6:1.
The common retinal findings of TA are dilation and tortuosity of retinal veins, microaneurysms, arteriovenous shunts, retinal neovascularisation and optic atrophy. Amaurosis fugax occurs in 8% of patients is believed to be a pre-AION feature and could be an indication to initiate corticosteroid treatment. Our Medline search led to only 3 case reports of AION as a presenting feature of TA. Our patient fulfilled 3 of 6 criteria defined by ’The American College of Rheumatology 1990’ [Table:1].
We suggest that young patients presenting with bilateral AION should also be investigated for Takayasu disease by fluorescein angiography and aortography since early medical treatment with corticosteroids could save vision in these patients.
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