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Year : 2014  |  Volume : 62  |  Issue : 9  |  Page : 951-952

Systemic lupus erythematosus retinopathy in a 32-year-old female: Report of a case

1 Shri Bhagwan Mahavir Vitreoretinal Services, Chennai, Tamil Nadu, India
2 Department of Uveitis, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Submission26-Nov-2013
Date of Acceptance04-Jun-2014
Date of Web Publication4-Nov-2014

Correspondence Address:
Pukhraj Rishi
Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18 College Road, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.143940

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Keywords: Systemic lupus erthematosus, SLE- retinopathy

How to cite this article:
Bhojwani D, Rishi E, Majumder PD, Saurabh K, Rishi P. Systemic lupus erythematosus retinopathy in a 32-year-old female: Report of a case . Indian J Ophthalmol 2014;62:951-2

How to cite this URL:
Bhojwani D, Rishi E, Majumder PD, Saurabh K, Rishi P. Systemic lupus erythematosus retinopathy in a 32-year-old female: Report of a case . Indian J Ophthalmol [serial online] 2014 [cited 2020 Aug 14];62:951-2. Available from: http://www.ijo.in/text.asp?2014/62/9/951/143940

Systemic lupus erythematosus (SLE) is rare in India with a prevalence of 3 in 100,000. [1] SLE retinopathy is second most common ocular manifestation after keratoconjunctivitis sicca and a major vision-threatening complication of SLE with an incidence of upto 29% in patients with systemic active disease. [2]

We present this photo essay to illustrate the classic features of SLE retinopathy and the co-ordinated role of Ophthalmologist (including sub-specialties) with Physicians (Rheumatologist and/or Dermatologist) in managing these multi-system and multi-organ autoimmune disorders.

  Case Report Top

A 32-year-old female patient presented with diminution of vision in both eyes since 3 months. She had a history of miscarriage in the first trimester, 1 year back. Physical examination revealed malar rash and discoid skin rash on the trunk and extremities [Figure 1]. Best corrected visual acuity was 20/200 in right and 20/40 in the left eye. Biomicroscopic examination of anterior segment, intraocular pressure and Schirmer's test were within the normal limits in both eyes. Fundus examination revealed cotton-wool spots, multiple large-vessel branch retinal artery occlusion (BRAO) with macular ischemia in right eye and superotemporal BRAO sparing macula in left eye [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]. She was referred to Rheumatologist and Dermatologist who diagnosed her with SLE. Laboratory investigations were positive for antinuclear antibodies and antidouble stranded/native DNA antibodies. Rest of her systemic investigations including anti-phospholipid antibodies were negative. Treatment was initiated with oral azathioprine, hydroxychloroquine, and steroids.
Figure 1: External photograph demonstrating malar rash on face, discoid skin rash over extensor surface of upper limbs with sparing of nails

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Figure 2: Right eye fundus montage photo showing pale optic disc, multiple large vessel branch retinal artery occlusions, vessel sheathing and cotton wool spots

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Figure 3: Right eye fundus fluoroscein angiography montage photo showing multiple large arteriole occlusions with macular ischemia

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Figure 4: Left eye fundus montage photograph showing multiple cotton wool spots scattered over posterior pole

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Figure 5: Left eye fundus angiography montage photograph showing supero-temporal arterioles occlusion (arrow heads) with macular sparing

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Figure 6: Spectral domain-optical coherence tomography image with raster line scan running through fovea demonstrates foveal thinning in right eye attributable to macular branch retinal artery occlusion (foveal thickness = 114 μ). Left eye scan demonstrates shadowing (nasal side) secondary to extensive cotton wool spots (foveal thickness = 164 μ)

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  Discussion Top

Retinopathy is an important manifestation of SLE, which develops with an incidence of 3-29%. [3] SLE retinopathy points to active lupus, anti-phospholipid antibody syndrome (APS), central nervous system lupus or drug-induced. Fundus examination is important because ocular fundus is the only part of the human body where small vessels can be directly visualized in a noninvasive manner. Life-table survival estimates have shown decreased survival in patients with SLE retinopathy, compared to SLE patients without retinopathy. [3]

A characteristic finding of lupus retinopathy is vasculitis of retinal capillaries associated with local microinfarction. [4] Large retinal vessel occlusions (central or branch; vein or arteriole) are more common with APS associated with SLE. [5] The mainstay of treatment is systemic immune-suppression.

Even though, ocular manifestations do not constitute the diagnostic criteria for SLE, they accurately indicate active systemic lupus; occult or overt. Early diagnosis, prompt referral, systemic immune-suppression and co-ordinated treatment strategies involving sub- multidisciplinary ophthalmologists, rheumatologist, and dermatologist play a key role in reducing ocular and systemic morbidity associated with SLE.

  References Top

Kumar A. Indian guidelines on the management of SLE. Indian J Rheumatol 2002;10:80-96.  Back to cited text no. 1
Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: A review of the literature. Autoimmune Dis 2012;290898.  Back to cited text no. 2
Foster CS. Systemic lupus erythematosus. In: Albert DM, Jacobiec FA, Azar DT, Gragoudas ES, Power SM, Robinson NL, editors. Principles and Practice of Ophthalmology. 2 nd ed. Pennsylvania: W.B Saunders Company; 2000. p. 4563-9.  Back to cited text no. 3
Anaya JM, Talal N. Head and neck findings in SLE: Sjögren's syndrome and the eye, ear, and larynx. In: Wallace DJ, Hahn BH, editors. Dubois' Lupus Erythematosus. 5 th ed. Baltimore: Williams and Wilkins; 1997. p. 783-91.  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


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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