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Photo Essay

Systemic lupus erythematosus retinopathy in a 32-year-old female

Report of a case

Bhojwani, Deepak; Rishi, Ekta; Majumder, Parthapartim Dutta; Saurabh, Kumar; Rishi, Pukhraj1,

Author Information
Indian Journal of Ophthalmology: September 2014 - Volume 62 - Issue 9 - p 951-952
doi: 10.4103/0301-4738.143940
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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

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 [Fig. 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 [Figs. 26]. 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
Figure 1:
External photograph demonstrating malar rash on face, discoid skin rash over extensor surface of upper limbs with sparing of nails
Figure 2
Figure 2:
Right eye fundus montage photo showing pale optic disc, multiple large vessel branch retinal artery occlusions, vessel sheathing and cotton wool spots
Figure 3
Figure 3:
Right eye fundus fluoroscein angiography montage photo showing multiple large arteriole occlusions with macular ischemia
Figure 4
Figure 4:
Left eye fundus montage photograph showing multiple cotton wool spots scattered over posterior pole
Figure 5
Figure 5:
Left eye fundus angiography montage photograph showing supero-temporal arterioles occlusion (arrow heads) with macular sparing
Figure 6
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)

Discussion

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.

1. Kumar A. Indian guidelines on the management of SLE Indian J Rheumatol. 2002;10:80–96
2. Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: A review of the literature Autoimmune Dis. 2012:290898
3. Foster CSAlbert DM, Jacobiec FA, Azar DT, Gragoudas ES, Power SM, Robinson NL. Systemic lupus erythematosus Principles and Practice of Ophthalmology. 20002nd ed Pennsylvania W.B Saunders Company:4563–9
4. Anaya JM, Talal NWallace DJ, Hahn BH. Head and neck findings in SLE: Sjögren's syndrome and the eye, ear, and larynx Dubois’ Lupus Erythematosus. 19975th ed Baltimore Williams and Wilkins:783–91
5. 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

Source of Support: Nil.

Conflict of Interest: None declared.

Keywords:

Systemic lupus erthematosus; SLE- retinopathy

© 2014 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow