Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by multisystem involvement, including renal, neurological, cardiovascular, and ocular manifestations. Among its ocular complications, lupus retinopathy is the most common. Lupus retinopathy, referring specifically to retinal hemorrhages, cotton-wool spots, retinal edema, and vascular occlusions directly caused by SLE (excluding drug-induced forms such as chloroquine-induced retinopathy), occurs in approximately 10% to 29% of SLE patients (1). However, retinal vasculitis and optic neuritis remain rare but severe manifestations, often leading to significant visual impairment if left untreated (2). These complications typically arise in the context of high systemic disease activity and are associated with vascular inflammation, immune complex deposition, and autoantibody-mediated endothelial dysfunction (3, 4).

Retinal vasculitis in SLE is often characterized by perivascular inflammation, vascular leakage, and potential occlusion, leading to ischemic retinal damage and neovascularization (3, 5). Optic neuritis, on the other hand, results from inflammation of the optic nerve, leading to acute vision loss, dyschromatopsia, and relative afferent pupillary defects (6). These conditions pose significant diagnostic challenges, as their presentations can overlap with other autoimmune, infectious, and thrombotic disorders.

Early recognition and appropriate immunosuppressive treatment are crucial for preserving vision and preventing irreversible damage. Corticosteroids remain the mainstay of initial therapy, but refractory or severe cases may benefit from biologic therapies such as rituximab (7). In this report, we present a case of a young female with newly diagnosed SLE who developed concurrent retinal vasculitis and optic neuritis, successfully treated with corticosteroids and rituximab. A literature review is included to provide further insight into the epidemiology, pathophysiology, diagnosis, and treatment options for these rare but devastating ocular manifestations of SLE.

A 26-year-old woman presented with a one-month history of facial erythema and a 10-day history of progressive blurred vision. Initially, she noticed a symmetrical erythematous rash over her face but did not seek medical attention. Ten days prior to admission, she developed sudden bilateral vision loss without identifiable triggers. She reported no fever, chills, limb numbness, hair loss, dry eyes, dry mouth, Raynaud’s phenomenon, muscle pain or weakness, joint stiffness, or systemic symptoms such as abdominal pain or diarrhea.

She was evaluated at the local ophthalmic hospital and diagnosed with optic neuritis. She received three doses of intravenous methylprednisolone (500 mg/day) with minimal improvement in visual acuity. Two days later, she experienced intermittent headaches, palpitations, nausea, and vomiting, with systolic blood pressure peaking at 170 mmHg. These symptoms prompted her admission to our hospital for further evaluation.

On admission, her best corrected visual acuity was 0.6 in the right eye and 0.8 in the left eye. Fundoscopic examination revealed bilateral optic disc edema with soft exudates, findings consistent with concurrent retinal vasculitis ( Figure 1a ). Enhanced vascular reflex is noted in the superior temporal retinal vessels, with scattered hemorrhagic spots. Scattered white exudates are observed in the posterior pole of the retina. Bilateral OCT scans of the optic nerve fiber layer (RNFL) thickness on admission reveal thinning of the nasal retinal nerve fiber layer and thickening of the temporal retinal nerve fiber layer ( Figure 2 ). Her blood pressure was elevated, and physical examination showed a butterfly rash across her face. However, there was no alopecia, joint swelling, or other cutaneous abnormalities.

Laboratory investigations revealed leukopenia with a white blood cell count of 1.51 × 10⁹/L, while routine liver and kidney function tests were normal. Inflammatory markers showed a serum amyloid A level of 12.45 mg/L and an erythrocyte sedimentation rate (ESR) of 23 mm/h. Autoantibody testing revealed a nuclear homogeneous pattern on ANA with a titer of 1:3200 and strongly positive anti-dsDNA antibodies at 188.73 IU/mL. Additional positive findings included antibodies against ds-DNA, nucleosome, histone, U1-snRNP, SS-A/Ro52, SS-A/Ro60, SS-B/La, and AMA-M2. Complement levels were significantly reduced, with C3 at 0.46 g/L and C4 at 0.07 g/L. Immunoglobulin G was elevated at 18.00 g/L. Other tests, including antineutrophil cytoplasmic antibodies (ANCA), lupus anticoagulant, antiphospholipid antibodies, anticardiolipin antibodies, C-reactive protein, ferritin, and 24-hour urine protein quantification, were within normal limits. The patient tested negative for infectious diseases such as tuberculosis and hepatitis B. Imaging studies further supported the diagnosis. Cranial magnetic resonance angiography (MRA) demonstrated vascular abnormalities consistent with secondary cerebral vasculitis ( Figure 3 ). Ultrasonography of the heart, abdomen, and other systemic evaluations revealed no abnormalities. Transient hypertension observed was likely due to acute inflammatory response and corticosteroid administration. Renal function tests and proteinuria assessments were normal, ruling out active lupus nephritis. Cranial imaging (MRI/MRA) excluded posterior reversible encephalopathy syndrome (PRES). Due to the patient’s urgent admission and personal preference, fluorescein angiography and visual evoked potentials (VEP) were not performed initially. However, optic nerve OCT and cranial MRI/MRA clearly documented optic nerve inflammation and cerebral vasculitis.

Based on her clinical presentation, ophthalmic findings, strongly positive anti-dsDNA and other autoantibodies, reduced complement levels, and systemic symptoms, a diagnosis of SLE was made. The ocular findings of optic neuritis and retinal vasculitis were identified as severe complications of SLE. Differential diagnoses such as infectious etiologies (tuberculosis, syphilis), autoimmune disorders (granulomatosis with polyangiitis, neuromyelitis optica), and hypertensive retinopathy were considered and systematically excluded through laboratory tests and imaging. The patient was treated with intravenous methylprednisolone (80 mg/d), followed by two doses of intravenous Rituximab (500 mg on the 2nd day post-admission and two weeks after admission) to control systemic inflammation and immunologic activity. Significant improvement was noted: her visual acuity returned to 1.0 bilaterally, and the facial erythema resolved before discharge. Fundus photographs obtained on 12 days after admission. Persistent bilateral disc edema and retinal exudates ( Figure 1b ). Hydroxychloroquine initiation was temporarily deferred due to severe ocular involvement. Ophthalmology consultation advised postponing it to avoid confounding retinal assessments. Initiation is planned after confirming retinal stability at 6 months. A lower initial dose of mycophenolate mofetil (750 mg/day) was chosen due to the patient’s low body weight and gastrointestinal sensitivity, with gradual escalation to 1500 mg/day after three months.

The patient was discharged on the 15th day after admission. She was prescribed oral prednisolone (40 mg/d) and mycophenolate mofetil (750 mg/d) for maintenance therapy. Antihypertensive medications were briefly administered during hospitalization but discontinued prior to discharge. At three months post-discharge, the patient maintained clinical stability with successful corticosteroid tapering and absence of systemic disease activity. Blood pressure remained stable within normal range throughout the follow-up period. Follow-up fundoscopic examination demonstrated significant improvement in retinal findings. Scattered white exudates are observed in the posterior pole of the retina, with a significant reduction in exudates compared to previous examinations. ( Figure 1c ). Laboratory test indicators and disease activity show significant improvement compared with before treatment ( Table 1 ).

This case underscores the importance of early recognition and prompt immunosuppressive therapy in SLE-related ocular vasculitis and optic neuritis, particularly in severe cases requiring biologic intervention. The concurrence of retinal vasculitis and optic neuritis in SLE presents a diagnostic challenge due to overlapping clinical features with other autoimmune and infectious etiologies. The patient initially presented with ocular manifestations as the first clinical sign of SLE, significantly complicating both diagnosis and therapeutic management. To contextualize the findings, we compared the present case with 30 previously published cases of SLE-related severe ocular manifestations ( Tables 2 , 3 ). The dataset categorized these cases into three groups: SLE with both retinal vasculitis and optic neuritis (7 cases) (8–14), SLE with retinal vasculitis alone (10 cases) (7, 15–23), and SLE with optic neuritis alone (13 cases) (6, 24–33). Our case falls into the first category, which represents the most severe ocular involvement.

The present case exhibited highly active systemic lupus, with markedly elevated ds-DNA titers, low complement levels, and concurrent neurovascular involvement, as suggested by MRA findings. This aligns with trends observed in patients with both retinal vasculitis and optic neuritis, who frequently exhibited multisystem disease, including lupus nephritis and CNS lupus (20, 34) In contrast, patients with retinal vasculitis alone also had active SLE but tended to have fewer concurrent neuropsychiatric symptoms (19, 22). Retinal vasculitis in these cases was often the first sign of systemic lupus exacerbation. Patients with isolated optic neuritis had a more variable degree of systemic lupus activity. Some had isolated optic neuritis with minimal systemic manifestations, while others developed CNS lupus over time (35).

In terms of treatment, our patient received intravenous methylprednisolone followed by rituximab and mycophenolate mofetil, achieving significant visual recovery. This aligns with treatment approaches observed in previous reports of concurrent retinal vasculitis and optic neuritis in SLE, where corticosteroid monotherapy was typically inadequate, necessitating additional immunosuppressive agents such as cyclophosphamide or rituximab to effectively control disease activity and preserve vision (12–14).

Notably, therapeutic responses and prognoses differ substantially between lupus-associated retinal vasculitis and optic neuritis. Patients with isolated retinal vasculitis generally show good initial responses to corticosteroids alone; however, maintaining remission frequently requires additional long-term immunosuppression (e.g., azathioprine or mycophenolate mofetil) (15, 19, 22). Early and aggressive intervention usually results in favorable outcomes, although visual prognosis can vary significantly depending on the extent and rapidity of vascular occlusion. Those with limited vaso-occlusion often achieve better visual prognoses, whereas cases involving severe ischemic retinopathy may lead to permanent vision loss despite therapy (15, 16).

In contrast, patients presenting with lupus-associated optic neuritis tend to respond initially to high-dose corticosteroids, often with noticeable improvement in acute visual symptoms. However, unlike typical demyelinating optic neuritis (as seen in multiple sclerosis), visual recovery in lupus-related optic neuritis is frequently incomplete. Long-term outcomes tend to be less favorable, marked by partial visual recovery, progressive optic nerve atrophy, or recurrent episodes despite continued immunosuppressive therapy (6, 29–31, 33). Approximately one-third of these patients experience relapses, underscoring the difficulty of achieving sustained remission (30, 31).

Patients like ours, presenting concurrently with both retinal vasculitis and optic neuritis, generally experience the most severe ocular involvement and thus have the worst prognoses overall. Many previously reported cases resulted in irreversible visual impairment due to profound retinal ischemia or persistent optic nerve damage (9, 11, 12). However, our patient achieved remarkable visual recovery, making her one of the best responders within this severe subgroup. Nonetheless, the high risk of disease recurrence mandates ongoing, carefully tailored immunosuppressive management. Therefore, recognizing these distinct therapeutic responses and prognostic outcomes is critical for rheumatologists, ophthalmologists, and neurologists involved in managing ocular manifestations of SLE. Early, aggressive, and individualized immunosuppressive therapy—along with diligent monitoring—is essential to optimize long-term visual outcomes in these challenging cases.

This case highlights the need for rapid diagnosis and aggressive immunosuppression in severe SLE-related ocular disease. Given that retinal vasculitis often signals active systemic lupus, early recognition is crucial. The association with antiphospholipid antibodies suggests that anticoagulation may be beneficial in select cases to prevent further vaso-occlusive events (6, 27, 31). Additionally, biologic therapies such as rituximab is emerging as promising treatments for refractory disease (7, 22).

Moving forward, continued research is needed to establish standardized treatment protocols for these rare but vision-threatening complications. Our findings reinforce the value of a multidisciplinary approach, integrating rheumatologists, ophthalmologists, and neurologists to achieve optimal patient outcomes. This case contributes to the growing body of literature on SLE-related ocular disease, providing valuable comparative insights between a real-world case and previously documented cases. Future studies with larger cohorts and long-term follow-up are necessary to refine therapeutic strategies and improve patient prognosis.