Case Presentation: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can involve multiple organs, including the central nervous system (CNS). Neuropsychiatric systemic lupus erythematosus (NPSLE) refers to a spectrum of neurological and psychiatric symptoms such as cognitive dysfunction, psychosis, mood disorders, and seizures. Status epilepticus is a rare and severe presentation often associated with a poor prognosis. We present the case of a 24-year-old woman whose initial manifestation of NPSLE was status epilepticus, accompanied by multiorgan involvement.A 24-year-old woman presented to the emergency department after a witnessed generalized tonic-clonic seizure. Two weeks prior, she experienced flu-like symptoms and gastrointestinal upset, followed by recurrent headaches, neck pain, gait instability, and a fall. She had no prior personal or family history of epilepsy. On arrival, she was in status epilepticus and required intubation for airway protection. Vital signs showed hypertension and tachycardia, and physical examination revealed ongoing tonic-clonic activity. Laboratory findings included anemia, thrombocytopenia, mild leukocytosis, anion gap metabolic acidosis, acute kidney injury, Peripheral smear showed schistocytes. A CT head was negative for hemorrhage, but MRI Brain revealed gyriform hyperintensities in the temporal, occipital and parietal lobes, and bilateral pontine and midbrain signal abnormalities suggestive of encephalitis.The patient was started on anticonvulsants, IV dexamethasone, and empiric antibiotics for possible bacterial meningitis. Cerebrospinal fluid (CSF) analysis revealed elevated protein and glucose, and an increased IgG/albumin ratio. CSF viral panel for encephalitis and bacterial cultures were negative. Autoimmune workup revealed strongly positive antinuclear antibody, anti-double stranded DNA, anti-sjögren’s syndrome-related antigen A and B, and low complement levels. Positive direct antiglobulin testing confirmed autoimmune hemolytic anemia (AIHA). These findings, coupled with multiorgan involvement, led to the diagnosis of SLE with NPSLE.The patient improved with pulse dose steroids and she was additionally started on weekly rituximab infusions for four weeks. Due to worsening renal function mycophenolate mofetil (MMF) was initiated for suspected lupus nephritis. She developed further complications including pleural effusions requiring thoracentesis, pericardial effusions, hypertension, and aspiration pneumonia. Complement levels and anti-dsDNA showed gradual improvement, and repeat MRI Brain after four weeks revealed near-complete resolution of brain abnormalities. She was eventually discharged to a rehabilitation facility with a slow prednisone taper and MMF. A subsequent renal biopsy confirmed Grade III lupus nephritis, prompting a switch to belimumab with clinical and laboratory improvement.
Discussion: This case shows the complexity of diagnosing and managing SLE in an acute care setting with multidisciplinary collaboration. For hospitalists, it is important to maintain a high index of suspicion for SLE in patients presenting with unexplained seizures and systemic findings. Early recognition and timely initiation of immunosuppressive therapy is essential in improving outcomes.
Conclusions: The limited evidence guiding the management of NPSLE highlights a critical need for further research on standardized treatment approaches for NPSLE within hospital medicine.