Dear Editor, A 16-year-old girl, a known case of systemic lupus erythematosus (SLE), presented to our institute with altered sensorium and two episodes of seizures within the prior 2 days. She also had proptosis of bilateral eyes on examination. She was afebrile with normal vital parameters (pulse - 88/min, blood pressure - 108/64 mmHg, and respiratory rate - 18/min). Routine investigations showed proteinuria (protein - 209 mg/dL, creatinine - 70 mg/dL, and protein/creatinine ratio - 2.99), but other blood investigations including urea and creatinine were normal (urea - 16 mg/dL, creatinine - 0.9 mg/dL, and albumin - 1.9 d/dL). Her high-sensitivity C-reactive protein increased from 23.9 mg/L (with normal C3 and C4) 1 month back to 72.8 mg/L. Activated partial thromboplastin time (APTT) and APTT lupus anticoagulant test were normal. The anti-Cardiolipin antibodies (aCLA) immunoglobulin G (IgG)/IgM was 98.9/31.8, and beta-2 glycoprotein 1 IgG/IgM was 22.0/13. Renal biopsy showed Class IV lupus nephritis. She was evaluated with computed tomography (CT) scan which showed ill-defined hypodensities in bilateral cerebellar hemispheres and parietal lobes, hyperdense masses in the extraconal space superiorly in bilateral orbits [Figure 1a and b]. Magnetic resonance imaging (MRI) of the brain showed asymmetric hyperintensities in bilateral parietotemporal lobes and cerebellar white matter [Figure 1c-h] on T2/fluid-attenuated inversion recovery imaging without diffusion restriction. Orbital masses are T2 hypointense and T1 isointense [Figure 1f and g] without diffusion restriction superior to the superior rectus muscles, and proptosis of both eyes was noted. There was mucosal thickening [Figure 1g] of the bilateral ethmoid, sphenoid, and maxillary sinuses. The possibility of fungal sinusitis was thought of and she was started on broad-spectrum antifungal agents (intravenous injection of amphotericin for 15 days). Sinus scrapings from the sphenoethmoid sinuses and cerebrospinal fluid did not show any evidence of fungal infection. Hence, the possibility of bilateral orbital pseudotumor with posterior reversible encephalopathy syndrome (PRES) was considered and steroid (injection dexamethasone 8 mg and tapered over 1 week) was added. Follow-up MR imaging after 1 month showed all the lesions had almost resolved [Figure 2a-f]. The posterior reversible encephalopathy changes had resolved [Figure 2a, b and d]. The orbital masses were significantly reduced in size with very minimal residual soft tissue thickening [Figure 2c and e] without any recurrence or residual which at further follow-up after 2 months [Figure 2f].Figure 1: Computed tomography axial sections (a and b) showing bilateral hyperdense orbital lesions and bilateral cerebellar white matter hypodensities (arrows). Fluid attenuated inversion recovery (c and d) axial sections and T2 coronal (e) showing bilateral cerebellar, temporal and left parietal white matter hyperintensities. T2 coronal (f) T1-weighted (pre- and post-contrast [g and h]) sagittal images showing bilateral orbital T2 hypointense nonenhancing lesions (arrows). Bilateral maxillary and sphenoethmoid sinus mucosal thickening noted (c and f)Figure 2: Follow-up magnetic resonance imaging after 4 weeks using fluid-attenuated inversion recovery axial, T2 and T1 postcontrast coronal sections showing near-complete resolution of white matter hyperintensities (asterisks in a and arrows in b & d). The orbital masses have significnatly reduced in size with very minimal residual masses (arrows in c and e). No recurrence or residual lesion noted at further follow-up at 2 months (f)SLE is a chronic systemic connective tissue disorder affecting almost all body parts. Any structure in the eye may be affected due to inflammatory or thrombotic disease processes. Pseudotumor is the third-most common orbital pathology.[1] It is commonly unilateral disease but bilateral disease points to an underlying systemic disorder. It often results in significant morbidity including vision loss. PRES is associated with many conditions notable of which are hypertensive states, severe infections, autoimmune diseases, certain drug intake, transfusion-related, etc. The brain parenchymal changes resolve once the inciting cause is removed. Up to one-third of patients may develop ocular involvement and any part of the eye including the pathway of vision may be affected due to the inflammatory or associated vasculitic disease processes.[1] Usually, patients complain of painful eye movements, impaired vision, diplopia, and proptosis. The differentials to be considered include orbital cellulitis, lymphoma, myositis apart from cavernous sinus thrombosis, and thyroid orbitopathy. Overall, it constitutes about 6% of the lesions occurring in orbit. There is no sex or race predilection, it can be seen in any age group most of them occurring in middle-aged patients. It can result in significant morbidity including visual loss. The clinical presentation of pseudotumor depends on the amount of inflammation and the site of the structure involved.[2] It can usually be diagnosed based on the clinical and imaging (MRI or CT) features without necessitating the tissue biopsy in most cases. At CT, pseudotumor appears as iso-to-subtle hyperdense soft-tissue mass at variable sites in the orbit. When involving the extraocular muscles, the tendinous portion is also involved. On MRI, typically they appear hypointense to the extraocular muscles on T2-weighted imaging due to the fibrous contents of the lesions. On T1-weighted MRI, they appear isointense without any restriction on diffusion-weighted imaging which is used to differentiate them from the lymphoid masses. Contrast enhancement is variable. Mild cases of orbital inflammatory pseudotumor may spontaneously resolve. Moderate-to-severe disease with disturbances in visual acuity or field, raised intra-ocular pressure, and/or significant proptosis may warrant the use of steroids, cytotoxic drug treatment, or even radiotherapy. However, in severe refractory cases with large masses, decompression surgeries may be needed.[3] First report of proptosis as presenting feature in SLE was done by Brenner and Shock[4] in 1974. Siebert and Srinivasan[5] in 2004 referred to 10 such reported cases on proptosis as presentation in patients with SLE. They presented a 23 years old who had recurrent proptosis and was diagnosed to have SLE during the second episode of proptosis. Subsequently, few more reports have described the presentation of proptosis in SLE.[6-9] Amirlak and Narchi[7] described a 9-year-old girl presenting with proptosis as isolated feature. Escudero González et al.[8] reported an adult patient with SLE and proptosis who was resistant to steroids and immunosuppressants and subsequently treated with rituximab. The various extra-ocular muscles have been reported to be involved-lateral rectus,[10] medial rectus,[11] superior rectus, and oblique[12] – or even in combination – right medial and left inferior rectus[13] or even bilateral medial and lateral recti muscles.[14] The proptosis may be due to isolated orbital fat inflammation – panniculitis – without the involvement of muscles as reported by Ohsie et al.[9] There is no clarity on the duration of follow-up imaging in orbital pseudotumors to look for signs of resolution. Our case shows that the patient may have to be followed up after a short interval even as early as within a month either from the onset of the first symptoms or after the first imaging to look for the resolution. Orbital pseudotumor can be a presentation of SLE in young and may need imaging evaluation. MRI can accurately delineate the lesion and differentiate it from other causes of visual symptoms. The lesions may on occasion show rapid resolution as in our case. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.