Systemic lupus erythematosus (SLE) is a complex autoimmune condition known for its diverse clinical presentations. Neuropsychiatric SLE refers to neurological or psychiatric symptoms that arise once other potential causes have been ruled out. While hydrocephalus occurring in the context of SLE is exceedingly rare and its underlying mechanism remains elusive, its recognition is crucial for informed management decisions. A 20-year-old female patient, diagnosed with SLE, had been under our rheumatology clinic's care for two years. She recently presented with confusion, which emerged a year after her initial diagnosis. Brain tomography revealed obstructive hydrocephalus, and subsequent investigations ruled out other potential causes, linking the condition to her clinical SLE. After consulting with a neurosurgeon, a ventricular drainage catheter was inserted. In response to this hydrocephalus development, a regimen of pulse steroid therapy was administered. Following this, mycophenolate mofetil was prescribed as an immunosuppressant agent and her clinical condition was successfully stabilized.
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