Case Report

Anterior Ischemic Optic Neuropathy and Central Retinal Artery

Occlusion – A Rare Presentation in Takayasu Arteritis
Downloaded from http://journals.lww.com/tnoa by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW

Akkayasamy Kowsalya, Mano Aarthi VM, Mahesh Kumar

Neuro Ophthalmology Services, Aravind Eye Hospital, Madurai, Tamil Nadu, India
nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 02/17/2024

Abstract
Arteritic anterior ischemic optic neuropathy is a common ophthalmic manifestation of giant cell arteritis in elderly patients. Central retinal artery
occlusion and anterior ischemic optic neuropathy are relatively rare presentations in Takayasu arteritis. Our report describes a young Indian
woman with Takayasu disease who presented with central retinal artery occlusion in one eye followed by anterior ischemic optic neuropathy
in the fellow eye leading to bilateral loss of vision. Prompt initiation of corticosteroid therapy resulted in visual recovery.

Keywords: Anterior ischemic optic neuropathy, central retinal artery occlusion, pulse corticosteroid, Takayasu arteritis

Introduction
Takayasu arteritis is a chronic granulomatous vasculitis of
large and medium sized arteries. Ocular involvement occurs in
45%.[1,2] Arteritic anterior ischemic optic neuropathy (AION)
is a rare presentation.[2‑5]
Case Report
Twenty nine year old woman presented with sudden painless
loss of vision in right eye for one day duration. She also
complained of intermittent jaw and lower limb claudication.
She gave history of acute visual loss two months back in the left
eye, which was diagnosed and treated as central retinal artery
occlusion (CRAO) with no visual recovery. She had Takayasu
disease for which she was on low dose oral corticosteroid and
methotrexate for three months.
Best corrected visual acuity (BCVA) was hand movements in
the right eye and no perception of light in the left eye. Pupils
were sluggish in the right eye (RE) and absolute afferent
pupillary defect in the left eye (LE). Intraocular pressure was
15 and 13 mm of Hg in right and left eye, respectively. Fundus
of the right eye [Figure 1] showed pallid optic disc oedema with
haemorrhages along margins, dilated and tortuous vessels. In
Received: 07‑02‑2023      Accepted in Revised Form: 21‑02‑2023
Published: 22-09-2023
Access this article online
Quick Response Code:
Website:
https://journals.lww.com/tnoa
the left eye, optic disc was pale with attenuated blood vessels
and pale background [Figure 2].
On systemic examination, peripheral pulses were feeble in
both carotid arteries and absent in brachial arteries bilaterally.
Systolic blood difference of 30 mmHg between the two upper
arms was observed.
Blood investigations revealed leucocytosis (20,100 cells/
cu.mm), raised erythrocyte sedimentation rate (ESR) at 1
hour (45 mm) and negative C‑reactive protein (CRP). Ocular
coherence tomography (OCT) of the right eye showed oedema
of the retinal layers surrounding the optic nerve head, ILM
folds with normal foveal contour and left eye OCT showed
atrophy of inner retinal layers, loss of foveal depression
with retinal thinning suggestive of old CRAO [Figure 3].
Two‑dimensional echocardiography was normal. CT
angiography [Figure 4] showed possibility of vasculitides
with involvement of ascending aorta, thoraco abdominal aorta,
complete occlusion of the left subclavian artery, moderate
occlusion of left common carotid and internal carotid arteries
and a mild stenosis of the right renal artery.
Address for correspondence: Dr. Mano Aarthi VM,
Neuro Ophthalmology Services, Aravind Eye Hospital, Madurai,
Tamil Nadu, India.
E‑mail: vm93man@yahoo.com
This is an open access journal, and articles are distributed under the terms of the Creative
Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to
remix, tweak, and build upon the work non‑commercially, as long as appropriate credit
is given and the new creations are licensed under the identical terms.
For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com

DOI: How to cite this article: Kowsalya A, Aarthi VM, Kumar M. Anterior ischemic
10.4103/tjosr.tjosr_14_23 optic neuropathy and central retinal artery occlusion – A rare presentation in
Takayasu arteritis. TNOA J Ophthalmic Sci Res 2023;61:365-7.

© 2023 TNOA Journal of Ophthalmic Science and Research | Published by Wolters Kluwer - Medknow 365
 Kowsalya, et al.: Anterior ischemic optic neuropathy in Takayasu arteritis
Downloaded from http://journals.lww.com/tnoa by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW
nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 02/17/2024
Figure 1: Fundus photograph of the right eye showing pallid edema
of the optic nerve head with dilated and tortuous vessels, superficial
haemorrhages along the disc margins
Figure 3: Optical coherence tomography of OD (a) and OS (b). (a) OCT of
OD showing ILM folds with other retinal layers being normal with normal
foveal contour. (b) OCT of OS showing atrophy of inner retinal layers with
loss of foveal contour suggestive of old central retinal artery occlusion
Our patient fulfilled four out of six criteria (age <29 years,
claudication of extremities, decreased brachial pulsations and
arteriogram abnormality) for the diagnosis of Takayasu arteritis
suggested by American College of Rheumatology.[6]
A pulse dose of intravenous methyl prednisolone injection
1 g/day was given for five days for treatment of AION.
Maintenance dose of oral corticosteroid T. Prednisolone 50 mg
was given and tapered. T. Methotrexate was stepped up to
20 mg once weekly along with folic acid supplementation.
During follow up, BCVA in the right eye improved from hand
movements to 5/60 (20/300) by day 5 and 6/24 (20/80) by
4 months. On 1 year follow up, her BCVA was 6/9 (20/30).
Ophthalmoscopy revealed pale optic disc in both eyes.
There were no further systemic complications. Aggressive
treatment with corticosteroids and stepping up of steroid
sparing agent resulted in visual recovery and remission of
the disease.
We report this case as the combination of CRAO and arteritic
AION in the setting of Takayasu arteritis is rare and to
366 TNOA Journal of Ophthalmic Science and Research ¦ Volume 61 ¦ Issue 3 ¦ July-September 2023
Figure 2: Fundus photograph of the left eye showing pale optic disc
suggestive of optic atrophy
Figure 4: CT angiography showing stenosis of both internal carotids, right
common carotid, aorta and right renal artery with complete occlusion of
the left subclavian artery
emphasise the importance of prompt initiation of aggressive
treatment with corticosteroids, which can prevent blindness.
Discussion
Arteritic AION most commonly occurs in old age (>50 years)
with giant cell arteritis (GCA). Arteritic AION in young patients
should raise suspicion of underlying vasculitic disorder apart
from GCA as it is extremely rare in the younger age group.
Other vasculitic causes of arteritic AION include disorders like
periarteritis nodosa, Wegener’s granulomatosis, Crohns disease,
rheumatoid arthritis, Behcet disease, ANCA vasculitis and
systemic lupus erythematosus. Takayasu disease with ophthalmic
manifestations of CRAO and arteritic AION is rare.[2‑5] Takayasu
arteritis presents as granulomatous vasculitis of large and
medium sized arteries. It most commonly involves the aorta and
its branches.[7] It usually occurs in second and third decades of
life and has a female predilection.[8] Autoimmune cell mediated
immunity is involved in the pathogenesis of this disorder.
Our patient was young and did not have features suggestive
of GCA or other vasculitic disorders. She also fulfilled the
 Kowsalya, et al.: Anterior ischemic optic neuropathy in Takayasu arteritis
American college of Rheumatology criteria for the diagnosis
of Takayasu arteritis with characteristic narrowing of aorta
and major arteries. Ocular manifestations occur only in a
small proportion of cases. Common fundus finding in this
disease is chronic ischemic retinopathy and hypertensive
retinopathy. [9] Systemic hypertension occurs secondary
to stenosis of vessels. AION is a rare presentation in this
Downloaded from http://journals.lww.com/tnoa by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AW
disease.[2‑5]
CRAO as an isolated finding and bilateral anterior ischemic
optic neuropathy as an initial presentation in Takayasu
disease have been reported.[4] Nithyanandam et al.[10] had
nYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 02/17/2024
reported a case of Takayasu arteritis, which manifested
with CRAO and subsequent AION. In their case report,
AION resulted due to self‑stoppage of steroids. Our patient
developed CRAO and AION despite treatment with systemic
corticosteroids and methotrexate, which resolved after
stepping up the dosage. Hence, adequate dosage of steroids
is necessary for remission.
Retinal artery occlusion and AION occur due to occlusion of
carotid artery, thereby decreasing the ocular perfusion and
resulting in ischemia. ESR and other acute phase reactants help
assess the activity of the disease. Aggressive treatment with
corticosteroids results in visual recovery. Long term treatment
is usually with steroid sparing agents like methotrexate,
cyclophophamide and biological agents.
This case is being reported for the rare combination of CRAO
and arteritic AION in Takayasu arteritis. Systemic examination
should be a routine even among ophthalmologists as it can be
lifesaving. Vascular occlusions in young patients are mostly
due to underlying systemic pathology.
Declaration of patient consent
The authors certify that they have obtained all appropriate
TNOA Journal of Ophthalmic Science and Research ¦ Volume 61 ¦ Issue 3 ¦ July-September 2023
patient consent forms. In the form, the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Chun YS, Park SJ, Park IK, Chung H, Lee J. The clinical and
ocularmanifestations of Takayasu arteritis. Retina 2001;21:132‑40.
2. Lewis JR, Glaser JS, Schatz NJ, Hutson DG. Pulseless (Takayasu)
disease withophthalmic manifestations. J ClinNeuroophthalmol
1993;13:242‑9.
3. Schmidt MH, Fox AJ, Nicolle DA. Bilateral anterior ischemic optic
neuropathyas a presentation of Takayasu’s disease. J Neuroophthalmol
1997;17:156‑61.
4. Malik KP, Kapoor K, Mehta A, Dadeya S, Guliani BP, Aggarwal S,
et al. Bilateral anterior ischaemic optic neuropathy in Takayasu arteritis.
Indian J Ophthalmol 2002;50:52‑4.
5. Karam EZ, Muci‑Mendoza R, Hedges TR 3rd. Retinal findings in
Takayasu’sarteritis. ActaOphthalmol Scand 1999;77:209‑13.
6. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH,
Edworthy SM, et al. The American College of Rheumatology 1990
criteria for the classification of Takayasu arteritis. Arthritis Rheum
1990;33:1129‑34.
7. Terao C, Yoshifuji H, Mimori T. Recent advances in Takayasu arteritis.
Int J Rheum Dis 2014;17:238‑47.
8. Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M,
et al. Kayasu arteritis. Ann Intern Med 1994;120:919‑29.
9. Wang WW, Ye JJ, Chen YX, Dai RP. Fundus manifestation and treatment
of Takayasu’s arteritis. Zhonghua Yan Ke Za Zhi 2012;48:124‑30.
10. Nithyanandam S, Mohan A, Sheth U. Anterior ischemic optic neuropathy
in a case of Takayasu’s arteritis. Oman J Ophthalmol 2010;3:94‑5.
367