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 Table of Contents  
Year : 2022  |  Volume : 17  |  Issue : 3  |  Page : 311-312

The eye of the wolf

P. D. Hinduja Hospital and Research Centre, Mumbai, Maharashtra, India

Date of Submission04-Jan-2022
Date of Acceptance22-Apr-2022
Date of Web Publication18-Aug-2022

Correspondence Address:
Dr. Sahana Baliga
No. 2nd Floor Wing No 4 OPD Building, P. D. Hinduja Hospital, Veer Savarkar Marg, Mahim, Mumbai - 400 016, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_3_22

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Systemic lupus erythematosus is a chronic multisystemic disease with ocular manifestations seen in up to 90% of patients during their disease course. Ophthalmic manifestations can be due to disease activity or complications of therapy. Detailed history and careful examination are essential to differentiate between the entities. Timely accurate diagnosis and prompt treatment can save vision. We report two patients, one with lupus activity and the other with ocular infection to bring out the nuances in the management of such patients.

Keywords: Neuropsychiatric systemic lupus erythematosus, ocular toxoplasmosis, posterior reversible encephalopathy syndrome, systemic lupus erythematosus

How to cite this article:
Baliga S, Mulkutkar S, Balakrishnan C. The eye of the wolf. Indian J Rheumatol 2022;17:311-2

How to cite this URL:
Baliga S, Mulkutkar S, Balakrishnan C. The eye of the wolf. Indian J Rheumatol [serial online] 2022 [cited 2022 Oct 2];17:311-2. Available from:

  Introduction Top

Systemic lupus erythematosus (SLE) is a multisystem disorder with ocular involvement in one-third of the patients.[1] Ocular infections are also reported, apart from the manifestations of the disease itself.[2],[3] We would like to present two patients with SLE to highlight the varied nature of ocular involvement in this disease.

Case 1

A 19-year-old girl was diagnosed with SLE. She had no ocular involvement and responded well to the initial regimen of oral prednisolone, hydroxychloroquine (HCQs), and azathioprine. While steroids and azathioprine were tapered off over the next 3 years, her disease activity was stable with HCQs of 300 mg/day.

Five years into the disease, she complained of sudden diminution of vision and the appearance of floaters in her right eye. She also had a “gray patch” in her field of vision. An initial assessment with the local ophthalmologist was that of unilateral optic disc edema with a large cotton wool spot. This was considered an extension of her primary disease process as SLE does have ophthalmic manifestations. She was advised intravenous steroids for 3 days; however, she opted for a second opinion. On a review of ophthalmology opinion, she was indeed found to have an ill-sustained rapid afferent pupillary defect of the right eye, mild blurring of the right optic disc, and a corroborating central scotoma on the visual field testing. Interestingly, fundoscopy showed macular retinitis and vitreous inflammation [Figure 1]a. The left eye was normal. Given the above findings, the initial diagnosis was revisited and she was treated along the lines of ocular toxoplasmosis. She was offered intravitreal clindamycin, which she refused. Hence, she was started on oral trimethoprim-sulfamethoxazole twice a day for the next 3 months supplemented with oral prednisolone at 0.5 mg/kg a few days later. She responded well to this treatment within the 1st week, and steroids were gradually tapered off. After 8 weeks of treatment, she recovered her vision, and a repeat fundoscopy showed significant improvement [Figure 1]b.
Figure 1: (a and b) Fundus picture of the right eye – toxoplasma retinochoroiditis

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Case 2

The second patient was a 32-year-old female who was initially diagnosed with inflammatory arthritis. After two years, she developed an intermittent low-grade fever with evening rise for 3 months, alopecia, abnormal behaviour, decreased appetite, oral ulcers, and weight loss of over 20 kgs in 6 months. Investigations confirmed the diagnosis of SLE and she was referred to our hospital for further management. She developed generalized convulsions on the 3rd day of in-patient admission and required intensive care. She was managed with antiepileptics but continued to have postictal confusion. Magnetic resonance imaging (MRI) brain showed bilateral patchy cortical/subcortical T2 hyperintensities in posterior temporoparietal-occipital regions, cerebellum, brainstem more on the right side, bilateral corona radiata and corpus callosum, all features that were consistent with posterior reversible encephalopathy syndrome (PRES). Intravenous methylprednisolone 1 g was initiated and continued for 5 days. She was shifted to intravenous rituximab 2 g given as 500 mg every week for 4 weeks. While her general sensorium gradually improved, her vision continued to be poor. Her pupillary reactions were normal. She could only perceive the movements of the hand. Fundoscopy revealed multiple cotton wool spots with mild attenuation of the vasculature suggesting lupus retinopathy. The fundus features would not account for her profound visual deficit. Hence, a diagnosis of cortical blindness secondary to the chronic occipital lesions due to PRES was made. With treatment, her visual acuity slowly improved and the fundus features improved over the next 4 weeks [Figure 2]. Repeat MRI brain has shown almost clearing of T2 hyperintensities compared to the earlier scans.
Figure 2: Resolution of cotton wool spots

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The above two cases highlight the diverse nature of the ocular involvement in SLE. The first patient was presumed to have optic disc edema and cotton wool spots as a manifestation of SLE. Revising the diagnosis to ocular toxoplasmosis and initiation of prompt treatment benefitted the young girl with the dramatic improvement of vision and resolution of the infection. Our second patient did have classical features of lupus retinopathy. She responded adequately to immunosuppression but without proportionate improvement in the vision, possibly due to cortical blindness secondary to chronic changes of PRES.

  Conclusion Top

Visual symptoms in lupus patients can be due to varied causes. A careful ophthalmological examination is required to distinguish an infectious pathology from the more common inflammatory process seen in SLE patients. Both conditions would require wide variations in treatment. The loss of vision in patients with active disease specifically those with neuropsychiatric SLE, cannot be solely attributed to lupus retinopathy. Improvement of vision may take longer in this subset of patients. Careful monitoring of visual acuity and fundoscopy throughout the treatment is mandatory. Early diagnosis and prompt treatment can prevent irreversible damage in patients with lupus retinopathy.[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: A review of the literature. Autoimmune Dis 2012;2012:290898.  Back to cited text no. 1
Rubin BR, DeHoratius RJ. Acute visual loss in systemic lupus erythematosus. J Am Osteopath Assoc 1989;89:73-7.  Back to cited text no. 2
Held R, Eckardt C. Bilateral traction detachment in necrotizing retinitis as a sequela of toxoplasmosis. Fortschr Ophthalmol 1990;87:206-8.  Back to cited text no. 3
Kwon S, Koo J, Lee S. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Pediatr Neurol 2001;24:361-4.  Back to cited text no. 4


  [Figure 1], [Figure 2]


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