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

From the Guidelines

Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a wide range of neurological and psychiatric manifestations, including headache, mood disorders, seizures, cognitive impairment, and cerebrovascular disease, as highlighted in the European League Against Rheumatism (EULAR) recommendations 1.

Key Manifestations and Recommendations

• The most frequent syndromes observed in NPSLE are headache, mood disorders, seizures, cognitive impairment, and cerebrovascular disease 1.
• Treatment of NPSLE depends on the specific symptoms but typically includes immunosuppressive medications, with hydroxychloroquine serving as the foundation of therapy for mild symptoms, and corticosteroids like prednisone and immunosuppressants such as cyclophosphamide, mycophenolate mofetil, or rituximab for more severe manifestations 1.
• Symptomatic treatments are also crucial, including antiseizure medications for seizures, antidepressants for mood disorders, and anticoagulation for patients with antiphospholipid antibodies and thrombotic events 1.
• Regular monitoring of disease activity through clinical assessment and sometimes MRI imaging is essential for managing NPSLE, as emphasized in the EULAR recommendations 1.

Diagnostic Approach

• The diagnostic work-up for suspected NPSLE should aim to exclude causes unrelated to SLE and may include cerebrospinal fluid analysis, EEG, neuropsychological tests, nerve conduction studies, and MRI 1.
• Attribution of neuropsychiatric manifestations to SLE, as opposed to non-SLE related causes, is critical and can be facilitated by considering risk factors, neuroimaging, and exclusion of confounding factors 1.

Treatment Principles

• Glucocorticoids and immunosuppressive therapy are indicated for NPSLE manifestations thought to reflect an inflammatory process, while antiplatelet/anticoagulants are recommended for atherothrombotic/antiphospholipid-related manifestations 1.
• Early diagnosis and aggressive treatment are crucial, as neurological involvement indicates more severe disease and can significantly impact quality of life 1.

Recent Guidelines

• The 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus emphasizes the importance of attributing neuropsychiatric manifestations to SLE and treating them accordingly, with a focus on glucocorticoids, immunosuppressive agents, and antiplatelet/anticoagulants as necessary 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 systems. Some of the key manifestations include:

• Neuropsychiatric symptoms such as cognitive impairment, headache, and psychiatric manifestations 2, 3, 4
• Central nervous system (CNS) involvement, which can include a range of symptoms such as seizures, psychosis, and cerebritis 2, 5
• Peripheral nervous system involvement, which can include symptoms such as neuropathy and myopathy 3, 6
• Antiphospholipid antibodies, which can increase the risk of neurological complications such as stroke and cognitive impairment 3

Diagnosis and Management

Diagnosing neurological manifestations of SLE can be challenging, and requires a comprehensive evaluation to rule out other potential causes 2, 5. Management of these manifestations typically involves a combination of treatments directed towards the neurological complication and therapies directed towards SLE itself 2, 3. Some of the treatment options include:

• High-dose glucocorticoids and intravenous cyclophosphamide for severe symptoms 2
• Rituximab, intravenous immunoglobulins, or plasmapheresis for patients who do not respond to initial treatment 2
• Azathioprine and mycophenolate for maintenance therapy or mild to moderate symptoms 2
• Anticoagulation and antiplatelet agents for patients with antiphospholipid antibodies or antiphospholipid syndrome 2, 3

Assessment and Evaluation

Assessing neurological involvement in SLE patients is crucial, and can be done using standardized questionnaires such as physician-administered and self-reported questionnaires 4. These questionnaires can help capture all possible neuropsychiatric syndromes and address specific symptoms such as headache, cognitive impairment, and psychiatric manifestations 4. Electromyography can also be used to diagnose peripheral nervous system involvement, such as acute axonal polyneuropathy 6.