Introduction
Seizures are life-threatening complications of neuropsychiatric systemic lupus erythematosus (NPSLE) and are often associated with poor outcomes. Cyclophosphamide immunotherapy is the mainstay of NPSLE treatment.
Case Presentation
A 52-year-old woman was admitted to the hospital with swollen hands and blurred vision in her left eye that had lasted for 6 months.
Medical History
- She had been diagnosed with systemic lupus erythematosus (SLE) and antiphospholipid syndrome in 2000, and her disease manifestations included lymphocytosis, lower limb numbness, decreased visual acuity and retinal artery occlusion.
- She took prednisone, warfarin and Tripterygium wilfordii, a traditional Chinese medicine, intermittently over the first few years after her diagnosis but had not taken any medication recently. She therefore visited hospital for further diagnosis and treatment.
Physical Examination
- Her vital signs were within normal limits, and heart, lung and abdomen examinations revealed normal findings.
- An examination of the extremities showed a scattered dark red rash on both lower limbs, and the fifth finger of the right hand was dark purple, but no lower limb oedema was observed.
- Neurological examination showed that the patient’s cognitive ability was decreased.
- Vision examination showed blurred left eye vision and her visual acuity in both eyes was decreased.
- She showed no abnormalities of muscle strength or a stiff neck with Kernig’s sign, and her deep tendon reflexes were symmetric.
- There were no signs of hair loss, photosensitivity, or oral ulcers.
Clinical Evaluation
- Laboratory test results showed a normal blood count. Stool and urine cultures were negative.
- The antinuclear antibody titre was 1/1000, with elevated IgG, anti-cardiolipin antibodies, lupus anticoagulant positivity and complement factor consumption.
- Magnetic resonance imaging (MRI) of the head revealed multiple ischaemic foci and lacunar cerebral infarction in both cerebral hemispheres, the demyelination of white matter and mild brain atrophy.
Treatment
- The patient was treated with methylprednisolone (MP; 40 mg intravenously once daily), warfarin (5 mg orally once a day), and hydroxychloroquine (200 mg orally twice a day), and she was started on cyclophosphamide 800 mg (600 mg/m2, approximately 15 mg/kg) on 19 May 2022. However, approximately 8-12 h after completing her first cyclophosphamide injection cycle to build up the dose, she vomited approximately 30 ml of blood and had a sudden, jerky movement of the limbs, along with cognitive dysfunction.
- She was immediately put on diazepam but still experienced three episodes of a tonic–clonic seizure. Her oxygen saturation gradually decreased and remained at 70% (8 L/m oxygen intake).
- Laboratory tests showed that routine blood counts and liver and kidney function parameters were within normal limits. She was urgently transferred to the intensive care unit for endotracheal intubation, with an invasive ventilator used for high-flow oxygen inhalation, and she received continuous sodium valproate injection for antiepileptic treatment as well as other symptomatic support treatment.
- MRI of the head found no additional abnormalities. She was discharged on the same continued medication of prednisone, warfarin, hydroxychloroquine, and sodium valproate with advice on regular follow-up and medication.
Follow up
On follow up she reported no occurrence of seizures thereafter.