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Year : 2022  |  Volume : 4  |  Issue : 1  |  Page : 50

Visual loss due to bilateral total cataract in Takayasu's arteritis in a young female

Department of Ophthalmology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission16-Jul-2022
Date of Decision05-Sep-2022
Date of Acceptance16-Sep-2022
Date of Web Publication28-Oct-2022

Correspondence Address:
Sucheta Parija
Department of Ophthalmology, All India Institute of Medical Sciences, Sijua, Patrapada, Bhubaneswar - 751 019, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_37_22

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Takayasu's arteritis (TKA) is a chronic, granulomatous, systemic vasculitis affecting the aorta and its branches. Ocular manifestations in patients with TKA play an important role in determining the visual prognosis. We describe a case of a young female with complaints of painless, progressive loss of vision in both eyes for 6 months who was clinically diagnosed with TKA. Her laboratory and radiological investigations confirmed the diagnosis. She had a bilateral cataract and operated for cataract in the right eye with guarded visual prognosis. This case report highlights the importance of creating awareness among ophthalmologists for early diagnosis and multidisciplinary management in cases of TKA for the prevention of blindness.

Keywords: Blindness, cardiology, cataract, Takayasu's arteritis

How to cite this article:
Parija S, Sharma G. Visual loss due to bilateral total cataract in Takayasu's arteritis in a young female. Pan Am J Ophthalmol 2022;4:50

How to cite this URL:
Parija S, Sharma G. Visual loss due to bilateral total cataract in Takayasu's arteritis in a young female. Pan Am J Ophthalmol [serial online] 2022 [cited 2023 Sep 27];4:50. Available from: https://www.thepajo.org/text.asp?2022/4/1/50/359845

  Introduction Top

Takayasu's arteritis (TKA) is a chronic, granulomatous, systemic vasculitis affecting the aorta and its branches, coronary and pulmonary artery leading to occlusion and stenosis of the involved arteries.[1] It predominantly affects women and occurs in second and third decades of life.[2] It was first described by a Japanese Ophthalmologist Mikito Takayasu in 1908 in a 21-year-old female with wreath-like appearance of blood vessels in the retina along with absent radial pulse.[3]

The prevalence of ocular manifestations in TKA is varied ranging from 8.1% to 68%.[4],[5],[6],[7] The signs of anterior segment ischemia including shallow anterior chamber, corneal edema, iris neovascularization, and glaucoma are seen along with posterior segment findings such as microaneurysms, cotton wool spots, small vessel dilatation, and retinal neovascularization.[8]

Although Takayasu's retinopathy and hypertensive retinopathy are primarily noted, cataract appears to be a rare ocular finding reported in 1%–2% of patients.[7],[8],[9] Here, we report a case of TKA with bilateral cataract and its multidisciplinary management.

  Case Report Top

A 21-year-old female presented with painless diminution of vision for 6 months. She was a diagnosed case of TKA in April 2020 and was on oral methotrexate 15 mg once weekly. In November 2020, she had an acute episode of cerebrovascular accident for which she was hospitalized and treated. In May 2021, she complained of a gradual decrease of vision in both eyes and was diagnosed with branch retinal vein occlusion by a local doctor.

On examination, she was conscious, thinly built, and weighed 30 kg. Her pulse rate was 78 beats/min, blood pressure of 100/70 mmHg in the right upper limb, and 90/60 mmHg in the left upper limb, and oxygen saturation of 98% in room air. Radial pulse was not detectable in both upper limbs and was recordable in lower limbs. Cardiac, neurological, and respiratory examination was normal.

Visual acuity was perception of light with inaccurate projection of light in both eyes. Pupils were fixed, mid-dilated, and nonreactive to light in both eyes. The anterior chamber was quiet. A total cataract was noted in both eyes [Figure 1]. Intraocular pressure was 14 mmHg in both eyes. B scan ultrasonography was normal [Figure 2]. A diagnosis of bilateral cataract secondary to TKA was made and physician consultation advised.
Figure 1: Ocular examination shows mature cataract with mid-dilated pupils in both the eyes

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Figure 2: B scan ultrasonography showing anechoic vitreous with normal optic nerve shadow and retina well attached in both eyes

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Laboratory investigations for blood sugar, liver function test, kidney function test, thyroid profile, urine examination, and coagulation profile were within normal limits. Hemoglobin was 10.2 g/dl, erythrocyte sedimentation rate (ESR) was raised at 38, and C-reactive protein (CRP) was raised at 25 mg/L. Serology for human immunodeficiency virus, hepatitis B and C, venereal disease research laboratory for syphilis, antinuclear antibody, and rheumatoid factor were negative. The Mantoux test was negative.

Her previous computerized tomography angiography of the head and neck revealed circumferential wall thickening with near-complete narrowing of left subclavian, left common carotid artery (CCA), brachiocephalic trunk, bilateral CCA, and coeliac trunk at origin. Magnetic resonance imaging of the brain showed acute infarct in the left anterior cerebral artery and middle cerebral artery (MCA) [Figure 3]. There were multiple infarcts, gliosis, encephalomalacia, and cortical necrosis suggestive of old MCA infarct [Figure 4]. Magnetic resonance angiography reported nonvisualization of the left internal carotid artery and faint visualization of the right internal carotid artery with significant narrowing of the posterior branch of the left MCA with a paucity of cortical branches [Figure 5].
Figure 3: MRI of the brain (diffusion-weighted MRI) showing diffusion restriction in the left MCA and ACA territory suggestive of an infarct (arrow mark). MRI: Magnetic resonance imaging, MCA: Middle cerebral artery, ACA: Anterior cerebral artery

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Figure 4: MRI of the brain showing T2/ FLAIR image suggestive of encephalomalacia changes, gliosis with ex vacuo dilatation of the left lateral ventricle. MRI: Magnetic resonance imaging

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Figure 5: MR angiography shows nonvisualization of the left internal carotid artery and significant narrowing of the posterior branch of the left MCA (arrow mark). MR: Magnetic resonance, MCA: Middle cerebral artery

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The patient was admitted to medical ward as she was in the acute phase of disease activity and was started on oral Cefixime 200 mg twice daily for 5 days and prednisolone 20 mg in tapering doses. Methotrexate 15 mg once weekly, folic acid 5 mg twice weekly, and baclofen 10 mg thrice daily were also administered orally. ESR and CRP were repeated every 72 h and it was showing a declining trend. She underwent an uneventful small incision cataract surgery in her right eye with no visual improvement and lost to follow-up.

  Discussion Top

Ocular changes in TKA are due to ischemia depending on the part of carotid arteries occluded, duration of ocular vascular insufficiency, and efficient functioning of collaterals in the eye.[4] There are case reports of retinal manifestations in the form of central or branch retinal artery occlusion, branch retinal vein occlusion, hypertensive retinopathy, and acute ischemic optic neuropathy.[10],[11],[12],[13],[14]

A cataract is rare and late presentation of TKA. Literature search using the words TKA, pulseless disease, cataract, retinopathy in PubMed, Embase, and Google scholar showed few case reports as described in [Table 1].[9],[15] Harada et al. reported a case of a 34-year-old female of TKA who had a poor visual outcome after cataract surgery.[9] Flores-Suarez et al. reported three cases of TKA in females aged 25–34 years presenting with complaints of diminution of vision in all cases and involving both the eyes in two cases. The symptoms were present for 5 months and had not received any treatment. Besides bilateral cataract, two of the patients had severe Takayasu's retinitis which the author's thought was due to ischemia that developed before the cataract.[15] The technique of cataract surgery did not differ from routine phacoemulsification but caution must be kept for intraoperative hyphaema despite no signs of iris and angle neovascularization.[16] Postoperative uveitis needs to be taken care by use of prednisolone acetate 1% eye drops tapered by 2 weeks and noninflammatory steroids eye drops to be continued for 3–4 weeks. There are few cases reported till date,[7],[9],[15],[16],[17] and our case was also a young female with bilateral cataract.
Table 1: Literature review of Takayasu arteritis cases with cataract

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There are multiple causes of cataracts in TKA. Long-term use of steroids in the treatment of TKA can be one of the reasons for steroid-induced cataract.[6] Harada et al. reported that due to severe ischemia there was excessive production of vascular endothelial growth factor (VEGF) which can lead to cataractous changes.[9] Some theories even suggested that alteration in the nutrition of lens due to circulatory disturbances, neuroreceptor damage, and anterior ischemic changes can also be attributing factors in the development of cataract.[15],[18] Visual prognosis after surgical intervention is poor due to Takayasu's retinopathy, retinal ischemia, and optic atrophy which was seen in our case.

This case report is unique in establishing the fact that although cataract is a rare in TKA, timely intervention is needed as they are young patients. TKA patients must undergo a routine ocular examination to detect the findings early and intervention in the form of laser photocoagulation, anti-VEGF injections, or surgery for retinopathy can help in restoring sight. A high index of suspicion and awareness among ophthalmologists is needed in the diagnosis and multidisciplinary management of this disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate 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


Conflicts of interest

There are no conflicts of interest.

  References Top

Conn DL, Hunder GG, Duffy JD. Vasculitic syndromes. In: Kelly WN, Harris ED, Ruddy S, Sledge CB, editors. Textbook of Rheumatology. 4thed. Philadelphia: WB Saunders; 1993. p. 1077-11.  Back to cited text no. 1
Panja M, Mondal PC. Current status of aortoarteritis in India. J Assoc Physicians India 2004;52:48-52.  Back to cited text no. 2
Uthman IW, Bizri AR, Hajj Ali RA, Nasr FW, Khalil IM. Takayasu's arteritis presenting as fever of unknown origin: Report of two cases and literature review. Semin Arthritis Rheum 1999;28:280-5.  Back to cited text no. 3
Chun YS, Park SJ, Park IK, Chung H, Lee J. The clinical and ocular manifestations of Takayasu arteritis. Retina 2001;21:132-40.  Back to cited text no. 4
Baba T, Itakura K, Tanaka R, Kawasaki T, Kiyosawa M, Numano F. Importance of fluorescein angiographic study in evaluating early retinal changes in Takayasu disease. Jpn J Ophthalmol 1999;43:546-52.  Back to cited text no. 5
Kiyosawa M, Baba T. Ophthalmological findings in patients with Takayasu disease. Int J Cardiol 1998;66 Suppl 1:S141-7.  Back to cited text no. 6
Sagar S, Kar S, Gupta A, Sharma BK. Ocular changes in Takayasu's arteritis in India. Jpn J Ophthalmol 1994;38:97-102.  Back to cited text no. 7
Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE. Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 1977;93:94-103.  Back to cited text no. 8
Harada M, Yoshida H, Mitsuyama K, Sakamoto M, Koga H, Matsuo K, et al. Aortitis syndrome (Takayasu's arteritis) with cataract and elevated serum level of vascular endothelial growth factor. Scand J Rheumatol 1998;27:78-9.  Back to cited text no. 9
Peter J, David S, Joseph G, Horo S, Danda D, Peter JV. Rare retinal manifestations in Takayasu arteritis. J Rheumatol 2013;40:1404-5.  Back to cited text no. 10
Noel N, Butel N, Le Hoang P, Koskas F, Costedoat-Chalumeau N, Wechsler B, et al. Small vessel involvement in Takayasu's arteritis. Autoimmun Rev 2013;12:355-62.  Back to cited text no. 11
Conrath J, Hadjadj E, Serratrice J, Ridings B. Branch retinal vein occlusion reveals Takayasu's arteritis. J Fr Ophtalmol 2004;27:162-5.  Back to cited text no. 12
Peter J, David S, Danda D, Peter JV, Horo S, Joseph G. Ocular manifestations of Takayasu arteritis: A cross-sectional study. Retina 2011;31:1170-8.  Back to cited text no. 13
Peter J, David S, Joseph G, Horo S, Danda D. Hypoperfusive and hypertensive ocular manifestations in Takayasu arteritis. Clin Ophthalmol 2010;4:1173-6.  Back to cited text no. 14
Flores-Suarez LF, Simon JA, Reyes PA, Soto ME, Castanon C, Navarro P. Takayasu's arteritis presenting as bilateral cataracts: Report of three cases. Rheumatology (Oxford) 2003;42:1005-7.  Back to cited text no. 15
Bassi ST, Natarajan V, Rishi E . An Ischemic Elegy Turned into Hemorrhagic Clamour -Cataract Surgery in Takayasu Arteritis. J Clin Exp Opthamol 2018; 9: 730.   Back to cited text no. 16
Das M K,, Pokharna R, Maikap D, Panda S. Ultra-widefield imaging in Takayasu arteritis. Indian J Ophthalmol: 2022; 70 ( 7): 2678-2679.   Back to cited text no. 17
Aiello LP, Avery RL, Arrigg PG, Keyt BA, Jampel HD, Shah ST, et al. Vascular endothelial growth factor in ocular fluid of patients with diabetic retinopathy and other retinal disorders. N Engl J Med 1994;331:1480-7.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1]


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