What are the neurological manifestations of Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Neuropsychiatric lupus, also called lupus affecting the nervous system, occurs when systemic lupus erythematosus (SLE) impacts the brain, spinal cord, or nerves, and its manifestations can include cerebrovascular disease, seizures, cognitive dysfunction, major depression, acute confusional state, peripheral nervous disorders, and psychosis, as reported in the EULAR recommendations for the management of SLE with neuropsychiatric manifestations 1. The neurological manifestations of SLE can be diverse and complex, and may require a multifaceted approach to treatment.

• Common manifestations include:
  • Cerebrovascular disease (CVD)
  • Seizures
  • Cognitive dysfunction
  • Major depression
  • Acute confusional state (ACS)
  • Peripheral nervous disorders
  • Psychosis
• The diagnostic work-up of suspected neuropsychiatric SLE is comparable to that in patients without SLE who present with the same manifestations, and aims to exclude causes unrelated to SLE, as stated in the EULAR recommendations 1.
• Treatment typically involves corticosteroids like prednisone (starting at 0.5-1 mg/kg/day) to reduce inflammation, often combined with immunosuppressants such as cyclophosphamide (500-1000 mg/m² monthly), mycophenolate mofetil (1-3 g/day), or rituximab (375 mg/m² weekly for 4 weeks) for severe cases, as recommended in the EULAR guidelines 1.
• Hydroxychloroquine (200-400 mg daily) serves as a foundation therapy for most lupus patients, helping prevent flares and reducing overall disease activity, as noted in the study by 1.
• Symptom-specific treatments are also important: anticonvulsants for seizures, antipsychotics for psychosis, and antidepressants for mood disorders, as mentioned in the EULAR recommendations 1.
• Regular monitoring of neurological symptoms, medication side effects, and disease activity is essential, as emphasized in the study by 1. This multifaceted approach is necessary because neuropsychiatric lupus can manifest in various ways, from headaches and cognitive dysfunction to seizures and stroke-like symptoms, resulting from direct immune-mediated damage to neural tissue, vascular inflammation, or antibodies crossing the blood-brain barrier, as discussed in the study by 1.

From the Research

Neurological Manifestations of Systemic Lupus Erythematosus (SLE)

The neurological manifestations of SLE can be diverse and affect both the central and peripheral nervous system. Some of the key manifestations include:

• Neuropsychiatric symptoms such as headaches, seizures, and psychosis 2
• Peripheral neuropathy, which can be associated with pain, numbness, and weakness in the affected areas 3
• Central nervous system involvement, including cerebritis, cerebellar ataxia, and transverse myelitis 2

Treatment of Neurological Manifestations

The treatment of neurological manifestations of SLE depends on the severity and type of manifestation. Some of the common treatments include:

• High-dose glucocorticoids and immunosuppressive agents such as cyclophosphamide, azathioprine, and mycophenolate mofetil for severe symptoms 2, 4
• Rituximab, intravenous immunoglobulins, or plasmapheresis for patients who do not respond to initial treatment 2
• Anticoagulation and antiplatelet agents for patients with thrombotic manifestations 2
• Azathioprine and mycophenolate mofetil for maintenance therapy in patients with mild to moderate symptoms 2, 4

Challenges in Treatment

There are several challenges in the treatment of neurological manifestations of SLE, including:

• Lack of standardized treatment protocols 3
• Limited evidence from clinical trials, with most data coming from case reports and case series 3
• Need for individualized treatment approaches based on the specific manifestation and severity of symptoms 2