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

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. 2–6]. 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

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 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)

Figure 3.

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

Figure 4.

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

Figure 5.

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

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.