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Year : 2021  |  Volume : 59  |  Issue : 2  |  Page : 172-174

Severe ischemic retinopathy with vasculitis and role of multimodal imaging in asymptomatic patient with systemic lupus erythematosus

Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India

Date of Submission05-Oct-2020
Date of Decision27-Oct-2020
Date of Acceptance01-Nov-2020
Date of Web Publication24-Jun-2021

Correspondence Address:
Dr. Vinit Jayendra Shah
Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_151_20

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Systemic lupus erythematosus (SLE) is a systemic disease with multiorgan involvement including eyes. We report a 37-year-old female, a known SLE case for 4 years, who came to our clinic to screen for hydroxychloroquine toxicity. Fundus showed changes of ischemic retinopathy, confirmed on optical coherence tomography angiography and fundus fluorescein angiography. Our case illustrates the importance of multimodal imaging and its correlation with systemic disease activity in SLE patients.

Keywords: Fundus fluorescein angiography, optical coherence tomography angiography, systemic lupus erythematosus retinopathy, systemic lupus erythematosus, vasculitis

How to cite this article:
Talari S, Shah VJ, Khadar SM, Adeel SS, Nair S. Severe ischemic retinopathy with vasculitis and role of multimodal imaging in asymptomatic patient with systemic lupus erythematosus. TNOA J Ophthalmic Sci Res 2021;59:172-4

How to cite this URL:
Talari S, Shah VJ, Khadar SM, Adeel SS, Nair S. Severe ischemic retinopathy with vasculitis and role of multimodal imaging in asymptomatic patient with systemic lupus erythematosus. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Jul 27];59:172-4. Available from: https://www.tnoajosr.com/text.asp?2021/59/2/172/319273

  Introduction Top

Systemic lupus erythematosus (SLE) is an autoimmune, chronic disease, in which organs and cells undergo damage by tissue-binding autoantibodies and immune complexes in up to one-third of patients. SLE is known as women's disease because 90% of patients are women of childbearing age.[1] The prevalence of SLE in India is 3 in 100,000.[2] Severe retinal vaso-occlusive diseases have rarely been reported as a manifestation in patients with SLE. The purpose of this case report is to highlight the importance of multimodal imaging in SLE patients, especially in a patient without any visual disturbances. It also demonstrates the ocular vascular events that reveal the disease and its diagnostic significance in young people which may threaten vision.

  Case Report Top

A 37-year-old female referred to us by a rheumatologist for the screening of hydroxychloroquine (HCQ) toxicity. She has been suffering from joint pains, skin rash, and is on oral HCQ 200 mg and oral prednisolone 10 mg for the past 4 years. The best-corrected visual acuity was 20/20 in both eyes. Slit-lamp examination of the anterior segment, intraocular pressure, and Schirmer's test was within the normal limits in both the eyes. The fundus examination revealed cotton-wool spots, intraretinal hemorrhages, and vasculitis in both the eyes [Figure 1]. Optical coherence tomography (OCT) showed retinal thinning and intraretinal hyperreflective spots suggestive of cotton wool spots [Figure 2]a and [Figure 2]b, and OCT-Angiography showed capillary nonperfusion areas in the macular area indicating ischemia in both the eyes [Figure 2]c and [Figure 2]d. Fundus fluorescein angiography (FFA) showed extensive capillary nonperfusion areas indicative of ischemia with vascular staining in both eyes, more in the right eye [Figure 3]. The patient was advised scatter photocoagulation in both the eyes. Investigations including complete blood picture, blood urea, serum creatinine, antiphospholipid antibodies, liver function tests, chest X-ray, and cardiac workup were within normal limits. Mantoux test, HIV, and hepatitis B surface antigen were nonreactive. C-reactive protein and erythrocyte sedimentation rate were raised. Color vision and central fields were normal ruling out HCQ toxicity.
Figure 1: Color fundus photograph of both eyes showing cotton wool spots, intraretinal hemorrhages, and few hard exudates at the macula

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Figure 2: (a and b) Optical coherence tomography image of both eyes showing retinal thinning and hyperreflective dots in the inner retinal layers. (c and d) Optical coherence tomography – angiography images of both the eyes showing extensive capillary dropout areas in the macula with normal foveal avascular zone

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Figure 3: Fundus fluorescein angiography montage of both eyes in the late phase showing extensive retinal ischemia and staining of vessels around the posterior pole and macular area suggestive of systemic lupus erythematosus retinopathy

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  Discussion Top

SLE is a chronic systemic autoimmune disorder that affects multiple organs by the deposition of autoimmune complexes. SLE can affect all the ocular and extraocular tissues. One-third of SLE patients have ocular manifestations that vary from patient to patient and are markers of systemic disease activity.[1] SLE's most common ocular manifestation is keratoconjunctivitis sicca, and second most common is SLE retinopathy (3%–29%).[2],[3] The retinal examination is vital in systemic vasculopathy because it is the only part of the body where small vessels can be directly visualized in a noninvasive manner. SLE retinopathy can be present in different forms; the most common is microangiopathy, similar to hypertensive and diabetic retinopathy. The earliest findings are cotton wool spots and retinal hemorrhages.[4] The vaso-occlusive form can present with widespread capillary nonperfusion areas and major vessel occlusion (central and branch retinal vasculature), leading to vision-threatening complications. The occlusive type is common when SLE is associated with antiphospholipid antibody syndrome.[5] Vasculitic form is uncommon, which presents as sheathing and cuffing of vessels and vaso-occlusion is the end point, which was seen in our case. SLE optic neuropathy and choroidopathy may present rarely.

According to the American College of Rheumatology, ocular manifestations are not a part of SLE diagnostic criteria. Even though ocular manifestations do not constitute SLE's diagnostic criteria, they accurately indicate active systemic lupus, and the vaso-occlusive retinopathy is associated with similar changes in the central nervous system vasculature.[6] Life-table survival estimates have shown decreased survival in SLE retinopathy patients, compared to SLE patients without retinopathy.[7] It emphasizes the need for regular fundus examination and multimodal imaging in all SLE patients.

Managing the posterior segment disease typically involves systemic immunosuppression with corticosteroids in an acute phase, followed by steroid-sparing immunosuppressive agents. Patients with severe vaso-occlusive disease, particularly with the presence of antiphospholipid antibodies, may benefit from additional anticoagulation and/or antiplatelet treatment.

In vasculitis associated with macular edema, local treatment with periocular corticosteroid injection or intravitreal antivascular endothelial growth factor injections may be useful.[8] In patients with profound retinal ischemia, panretinal photocoagulation is needed to address or prevent neovascularization, and vitrectomy surgery may be needed to treat complications such as vitreous hemorrhage and tractional retinal detachment.[9] Other therapies include plasmapheresis and plasma exchange.[8] Tolba et al., in their study, found that 40% of the patients had SLE retinopathy, and out of those eyes, 41.6% had normal fundus findings with retinopathy diagnosed only after FFA.[10] Hence, patients diagnosed with SLE should have a thorough ocular examination including FFA and OCT even with normal fundus findings.

In our case, we diagnosed extensive vaso-occlusive SLE retinopathy only after ocular examination and multimodal imaging in a patient referred to rule out HCQ toxicity, which required panretinal photocoagulation.

  Conclusion Top

The presence of SLE retinopathy is suggestive of high systemic disease activity. Early diagnosis, prompt referral, systemic immune suppression, and multidisciplinary approach, involving ophthalmologist, rheumatologist, and dermatologist, play a crucial role in reducing ocular and systemic morbidity associated with SLE. Regular ocular examination, along with multimodal imaging, has a significant role in the diagnosis and prevention of complications related to SLE retinopathy.

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

Kharel Sitaula R, Shah DN, Singh D. Role of lupus retinopathy in systemic lupus erythematosus. J Ophthalmic Inflamm Infect 2016;6:15.  Back to cited text no. 1
Bhojwani D, Rishi E, Majumder PD, Saurabh K, Rishi P. Systemic lupus erythematosus retinopathy in a 32-year-old female: Report of a case. Iandian J Ophthalmol 2014;62:951-2.  Back to cited text no. 2
Sobrin L, Foster CS. Systemic Lupus Erythematosus Choroidopathy BMJ, IV; 1994.  Back to cited text no. 3
Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: A review of the literature. Autoimmune Dis 2012;2012:290898.  Back to cited text no. 4
Ushiyama O, Ushiyama K, Koarada S, Tada Y, Suzuki N, Ohta A, et al. Retinal disease in patients with systemic lupus erythematosus. Ann Rheum Dis 2000;59:705-8.  Back to cited text no. 5
Jabs DA, Fine SL, Hochberg MC, Newman SA, Heiner GG, Stevens MB. Severe retinal vaso-occlusive disease in systemic lupus erythematous. Arch Ophthalmol 1986;104:558-63.  Back to cited text no. 6
Foster CS. Systemic lupus erythematosus. In: Albert DM, Jacobiec FA, Azar DT, Gragoudas ES, Power SM, Robinson NL, editors. Principles and Practice of Ophthalmology. 2nd ed. Pennsylvania: W. B Saunders Company; 2000. p. 4563-9.  Back to cited text no. 7
Song YH, Kim CG, Kim SD, Kim YY, Choe JY. Systemic lupus erythematosus presenting earlier as retinal vaso-occlusion. Korean J Intern Med 2001;16:210-3.  Back to cited text no. 8
Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 2007;46:1757-62.  Back to cited text no. 9
Tolba DA, El-Fayoumi DM, Abdelaziz MS, Nabih MH. Fluorescein angiographic findings in patients with active systemic lupus erythematosus. Ocul Immunol Inflamm 2017;25:884-90.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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