Systemic lupus erythematosus (SLE) is a multifaceted autoimmune disorder that can affect various organ systems, including the central nervous system (CNS), which can lead to neurologic and neuro-ophthalmic manifestations as well (10-30% of cases). Prompt recognition of CNS involvement is critical, as it is associated with increased mortality and often necessitates an elaborate and complex treatment regimen to manage effectively.

A 62-year-old right-handed female with history of SLE on prednisone, leflunomide, and mycophenolate, migraine, and central venous thrombosis presented to the Neuro-ophthalmology clinic with multiple episodes of abrupt transient bilateral vision loss, worsening headaches, photophobia, and scalp tenderness. The initial examination revealed a right homonymous visual field defect. MRI Brain identified a corresponding left occipital mass extending both supra- and infra-tentorially. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, and dural biopsies ultimately confirmed lupus cerebritis. Despite treatment with immunomodulatory agents, the patient’s symptoms worsened aggressively, with an increasing homonymous altitudinal field defect and progressive enlargement of the occipital mass on MRI. This led to the addition of rituximab along with high-dose steroids. However, her condition continued to decline, presenting with new-onset weakness and gait instability, necessitating hospitalization and pulse-steroid therapy. Although there was initial stabilization, her headaches and vision deteriorated, and a repeat MRI revealed further progression of the occipital mass, raising concerns for potential herniation. An interdisciplinary team decision was made to initiate fractionated radiotherapy. Two months later, the patient experienced significant symptomatic relief, and follow-up MRI showed a marked reduction in both the mass burden and meningeal involvement.

Progressive lupus cerebritis involving the occipital lobe in this case caused significant altitudinal visual loss, refractory to multiple anti-inflammatories and immunomodulators. Fractionated radiotherapy was initiated, showing impressive clinical improvement and brain lesion regression. Radiotherapy is a considerable option for patients with refractory lupus cerebritis.