From the Guidelines
Lupus encephalitis should be treated with high-dose corticosteroids, such as methylprednisolone 1000mg IV daily for 3-5 days, followed by oral prednisone 1-2mg/kg/day with gradual tapering, as recommended by the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus 1.
Treatment Approach
The treatment approach for lupus encephalitis involves a combination of immunosuppressive therapies and supportive care.
- High-dose corticosteroids are the first line of treatment, with the goal of reducing inflammation and preventing further neuronal damage.
- For severe or refractory cases, immunosuppressants like cyclophosphamide (500-1000mg/m² monthly), mycophenolate mofetil (1-3g daily), or rituximab (375mg/m² weekly for 4 weeks) may be added to the treatment regimen 1.
- Patients require close monitoring for seizures, which may need anticonvulsant therapy such as levetiracetam 500-1000mg twice daily.
- Hospitalization is usually necessary during acute episodes, with regular neurological assessments and brain imaging.
Diagnosis and Attribution
Diagnosis of lupus encephalitis involves a combination of clinical evaluation, laboratory tests, and imaging studies.
- Attribution to SLE—as opposed to non-SLE—related neuropsychiatric manifestations, is essential and can be facilitated by neuroimaging, investigation of cerebrospinal fluid, consideration of risk factors, and exclusion of confounding factors 1.
- The diagnostic work-up of suspected NPSLE is comparable to that in patients without SLE who present with the same manifestations, and aims to exclude causes unrelated to SLE 1.
Prevention of Flares and Maintenance Therapy
Prevention of flares and maintenance therapy are crucial in the management of lupus encephalitis.
- Treatment should aim at remission or low disease activity and prevention of flares in all organs, maintained with the lowest possible dose of glucocorticoids 1.
- Hydroxychloroquine is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight, as part of the maintenance therapy regimen 1.
- Long-term maintenance therapy is often needed to prevent recurrence of lupus encephalitis and other SLE-related manifestations.
From the Research
Definition and Symptoms of Lupus Encephalitis
- Lupus encephalitis is a condition characterized by inflammation of the brain in patients with systemic lupus erythematosus (SLE) 2
- Symptoms of lupus encephalitis include seizures, sensory aphasia, pseudobulbar affect, depression, anxiety, and hallucination 2
Treatment of Lupus Encephalitis
- High-dose glucocorticoids and intravenous cyclophosphamide therapy have been shown to be effective in treating lupus encephalitis 2
- Methylprednisolone pulses followed by high-dose prednisolone and intravenous cyclophosphamide can lead to rapid improvement in symptoms 2
- Cyclophosphamide remains the 'gold standard' treatment for severe organ-threatening SLE, including renal and central nervous system lupus 3
Role of Anti-Glutamate Receptor Antibodies
- Elevated anti-glutamate receptor antibodies (anti-GluRs) in serum and cerebrospinal fluid (CSF) have been associated with lupus encephalitis 2
- Reduction of anti-GluRs in serum or CSF may be a prognostic marker for treatment response 2
Treatment Approaches for SLE
- Intravenous methylprednisolone (IVMP) is widely used in clinical practice for treating SLE, but its precise role is still not well-defined 4
- Repeated pulses of methylprednisolone with reduced doses of prednisone may improve the outcome of lupus nephritis 5
- Cyclophosphamide with oral prednisone is more effective than oral prednisone alone in treating SLE 3