Systemic lupus erythematosus (SLE) is rare in India with a prevalence of 3 in 100,000. 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.
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.
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.
Retinopathy is an important manifestation of SLE, which develops with an incidence of 3-29%. 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.
A characteristic finding of lupus retinopathy is vasculitis of retinal capillaries associated with local microinfarction. Large retinal vessel occlusions (central or branch; vein or arteriole) are more common with APS associated with SLE. 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.
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Conflict of Interest: None declared.