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Year : 2010  |  Volume : 55  |  Issue : 3  |  Page : 268-270
A case to illustrate the role of ophthalmologist in systemic lupus erythematosus

1 Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India
2 Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India

Date of Web Publication25-Sep-2010

Correspondence Address:
D M Thappa
Department of Dermatology and STD, JIPMER, Pondicherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.70686

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Systemic lupus erythematosus (SLE) affects the eye as part of the disease or due to the drugs used in therapy. Ocular involvement is seen in one third of the patients with SLE. SLE is rare in India and found less frequently in males and children. SLE retinopathy is usually bilateral. We report an unusual case of unilateral macular infarction in a boy caused by systemic lupus erythematosus. A fourteen year old boy was presented with skin rashes and loss of vision in left eye. Posterior segment examination showed hyperemic edematous disc, arteriolar attenuation, venous dilatation, multiple cotton wool spots around the disc and macula in the left eye. There was no improvement in vision with pulse steroids and cyclophosphamide. The clinical implication of SLE retinopathy is that the disease is severe and warrants systemic immunosuppressive therapy. SLE-induced macular infarction is rare and has poor visual prognosis. As serious ocular complications of SLE can be silent, routine ophthalmological evaluation is warranted in all patients.

Keywords: Systemic lupus erythematosus, macular infarction, India, lupus retinopathy, unilateral

How to cite this article:
Rao VA, Pandian DG, Kasturi N, Muthukrishanan V, Thappa D M. A case to illustrate the role of ophthalmologist in systemic lupus erythematosus. Indian J Dermatol 2010;55:268-70

How to cite this URL:
Rao VA, Pandian DG, Kasturi N, Muthukrishanan V, Thappa D M. A case to illustrate the role of ophthalmologist in systemic lupus erythematosus. Indian J Dermatol [serial online] 2010 [cited 2022 Jun 28];55:268-70. Available from:

   Introduction Top

Systemic lupus erythematosus (SLE) is a chronic autoimmune multisystem disease wherein the eye is involved as a part of active lupus or antiphospholipid antibody syndrome or due to drugs used in the treatment of SLE. SLE is rare in India with a prevalence of 3 in 100, 000. [1] The median age of onset is 24.5 years and the sex ratio (F:M) is 11:1. [1] Majority of patients (90%) are female. [1] . Ocular manifestations affecting the eye or the visual system are seen in 25 - 33% of patients with SLE. [2],[3] Ocular involvement may precede or follow the systemic illness. SLE retinopathy is a common ocular manifestation secondary to keratoconjuctivitis sicca. [2],[4] About 10-20% of patients of SLE have a childhood onset of the disease. [5] SLE-induced macular infarction is uncommon and is usually bilateral. We report a case of sight threatening unilateral SLE retinopathy in a boy.

   Case Report Top

A fourteen-year-old boy presented with diminished vision in the left eye, fever, and headache for two weeks. He had hair loss over the occiput, discoid rashes over the face, forehead, trunk and both lower limbs that exacerbated in sun light [Figure 1]. He was a recently diagnosed case of SLE treated with hydroxychloroquine 20 mg/day. His visual acuity was 20/40 in the right eye and was hand movements close to face with accurate projection of rays in the left eye. Anterior segment examination was normal in both eyes and there was no relative afferent pupillary defect. Posterior segment examination showed hyperemic edematous disc, arteriolar attenuation, venous dilatation, multiple cotton wool spots around the, and macula with macular edema in the left eye. Right eye had no evidence of SLE retinopathy. In view of poor vision with extensive cotton-wool spots at the macula, absence of relative afferent pupillary defect, macular infarction of left eye was diagnosed.
Figure 1: Discoid rashes on face and trunk

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The investigation revealed that his erythrocyte sedimentation rate was elevated, 80 mm/hr. LE cells were positive in serum. Anti ds DNA and ANA tests were positive. His leucocyte count was within normal limits. Skin biopsy showed hyperkeratotic papillomatosis, follicular plugging, and dermis showed mild focal periadnexal inflammatory infiltrate. Renal parameters were within normal limits. Magnetic resonance imaging of brain was within normal limits.

Later he developed irrelevant speech, forgetfulness, alteration in behavior, hence CNS lupus was suspected. He also had palpable purpura, which disappeared in 2 days. He was started on antibiotics and pulse methyl prednisolone followed by a course of oral steroids. Single pulse cyclophosphamide provided symptomatic relief and reduced neuropsychiatric symptoms. This did not improve the vision in the left eye. There was no effect in the right eye in 4 months follow up. The left eye fundus findings, on follow up, were optic atrophy, macular scarring, sheathing of vessels and cotton wool spots [Figure 2]. The macular edema and cotton wool spots at macula regressed with residual macular scarring and optic atrophy.
Figure 2: Left eye fundus. Yellow arrow - Disc pallor (optic atrophy), Red arrow - vascular sheathing, Green arrow - Macular scarring, Black arrow - Cotton wool spot

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   Discussion Top

Cutaneous manifestations are seen in 85% of cases, which includes malar rash, discoid rash with photosensitivity and alopecia. [6] Other manifestations include arthritis, glomerulonephritis, serositis, hematological, and central nervous system (CNS) disturbances. [2],[6] Ocular manifestations are seen in one third of the cases with a form of discoid lupus erythematosus of the eyelids or episcleritis, scleritis or secondary Sjφgren's syndrome. It can also be sight threatening in the form of lupus retinopathy. [4]

The various pathogenic mechanisms implicated in SLE are immune complex deposition, vasculitis and thrombosis. [4] Histopathologicaly infiltration of vessel wall with fibrillar material causing vascular constriction and hyaline thrombus formation is seen [2,7] This manifests as cotton wool spots, retinal vein or artery occlusion and macular infarction. [3] It causes perivascular lymphocyte infiltration and the vessel wall is free of inflammatory cells, hence not causing a true vasculitis. [2] Antiphospholipid/antineuronal antibody-mediated cytotoxicity results in retinal cell death and optic nerve demyelination. [4]

Retinal affection occurs in 7-26% [3],[4] of SLE patients and are invariably bilateral. Lupus retinopathy may be asymptomatic or may result in permanent visual impairment. SLE-induced microvasculopathy is characterized by cotton wool spots, retinal hemorrhages, perivascular exudates and vascular tortousity. In patients positive for antiphospholipid antibody, vaso-occlusive conditions such as CRAO, CRVO, and BRVO are common. [7],[8] Choroidal involvement can manifest as subretinal fluid accumulation, retinal pigment epithelium detachments or central serous chorioretinopathy. [4] Toxic maculopathy can result from hydroxychloroquine therapy but only at a dose of >6.5 mg/kg/d for more than 5 years. [4] Ocular involvement in SLE is usually silent unless its complications cause blindness. Complications such as vitreous hemorrhage, tractional retinal detachment, secondary neovascular glaucoma, secondary to proliferative retinopathy and optic atrophy are rare but sight threatening. [4],[7] Routine ophthalmic referral can aid in early diagnosis and treatment with laser photocoagulation and vitrectomy, preventing visual loss in proliferative lupus retinopathy. [7]

Macular infarction can present as the initial manifestation of SLE. [9],[10] It causes marked loss of vision and rarely responds to high dose systemic corticosteroids or other immunosuppressive therapy and causes poor visual prognosis. [10] Macular infarction is not found to be correlated with elevated antiphospholipid antibody levels.

Scleritis and SLE retinopathy are hallmark of systemic disease activity and requires systemic immunosuppresion. [4] Treatment for SLE includes photoprotection, hydroxychloroquine, systemic steroids, immunosuppressive agents such as cyclosporine, azathioprine, chlorambucil, and plasmapheresis. Lupus retinopathy has been shown to correlate well with raised antiphospholipid antibody levels. [3] It has been shown that patients with SLE retinopathy are at a higher risk of developing CNS lupus (71%) when compared to those without retinopathy (13%). [2]

   Conclusion Top

The manifestations of SLE are diverse. Eye involvement may be sight threatening or an indicator of systemic disease activity and reflects systemic vascular damage. The serious ocular manifestation such as lupus retinopathy usually requires systemic immunosuppressant and antithrombotic agents in order to reduce the ocular morbidity associated with the disease. Hence, ophthalmic evaluation is mandatory for patients with SLE.

   References Top

1.Kumar A. Indian guidelines on the management of SLE. J Indian Rheumatol Assoc 2002;10:80-96.  Back to cited text no. 1      
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. 2nd ed. Pennsylvania: W.B Saunders company; 2000. p. 4563-9.  Back to cited text no. 2      
3.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. 3  [PUBMED]  [FULLTEXT]  
4.Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology 2007;5:1-6.  Back to cited text no. 4      
5.Font J, Cervera R, Espinosa G, Pallarιs L, Ramos-Casals M, Jimιnez S, et al. Systemic lupus erythematosus (SLE) in childhood:analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis 1998;57;456-9.  Back to cited text no. 5      
6.Uy HS and Chan PS. Systemic lupus erythematosus. In: Foster CS, Vitale AT editors. Diagnosis and treatment of uveitis. Pennsylvania:WB Saunders Company; 2002. p. 601-9.   Back to cited text no. 6      
7.Ho TY, Chung YM, Lee AF, Tsai CY. Severe vaso-occlusive retinopathy as the primary manifestation in a patient with systemic lupus erythematosus. J Chin Med Assoc 2008;71:377-80.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Durukan AH, Akar Y, Bayraktar MZ, Dinc A, Sahin OF. Combined retinal artery and vein occlusion in a patient with systemic lupus erythematosus and antiphospholipid syndrome. Can J Ophthalmol 2005;40:87-9.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Sahu DK. An unusual presentation of lupus retinopathy. Indian J Ophthalmol 2008;56:72-3.  Back to cited text no. 9  [PUBMED]  Medknow Journal  
10.Shein J, Shukla D, Reddy S, Yannuzzi LA, Cunningham ET. Macular infarction as a presenting sign of systemic lupus erythematosus. Retinal cases and brief reports 2008;2:55-60.  Back to cited text no. 10      


  [Figure 1], [Figure 2]

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