What are the guidelines for Neurological Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Neuropsychiatric lupus (NPSLE) management should prioritize a multidisciplinary approach, focusing on both symptom control and underlying disease activity, with treatment guided by the presumed underlying pathophysiological mechanism, either inflammatory or embolic/thrombotic/ischaemic, as recommended by the 2019 update of the EULAR recommendations 1.

Key Considerations

• The treatment approach should be individualized based on the specific manifestations of NPSLE and the presence of antiphospholipid antibodies.
• Glucocorticoids (GC) and/or immunosuppressive (IS) agents are considered for inflammatory mechanisms, while anticoagulant/antithrombotic treatment is favored when antiphospholipid antibodies are present.
• Combination therapy of IS and anticoagulant/antithrombotic agents may be necessary in cases where both pathophysiological processes coexist.

Diagnostic Work-Up

• Initial diagnostic work-up should be similar to that in non-SLE patients presenting with the same manifestations, aiming to exclude non-SLE-related causes 1.
• Investigations may include cerebrospinal fluid analysis, EEG, neuropsychological tests, nerve conduction studies, and MRI to assess brain structure and function.

Treatment Options

• First-line treatment typically includes corticosteroids for acute manifestations, with immunosuppressants added as steroid-sparing agents for severe or moderate symptoms.
• Symptomatic treatments, such as antiepileptics for seizures, antipsychotics for psychosis, and antidepressants for mood disorders, are also considered.
• Anticoagulation with warfarin (target INR 2-3) is indicated when antiphospholipid antibodies are present with thrombotic events, as supported by the EULAR recommendations 1.

Monitoring and Prevention

• Regular cognitive assessment, MRI monitoring, and cerebrospinal fluid analysis help track disease progression.
• Preventative strategies should be tailored according to the patient's antiphospholipid antibody status, infectious disease risk profile, and cardiovascular disease risk profile, as emphasized in the 2019 EULAR update 1.

From the Research

Guidelines for Neuro Lupus

• The diagnosis and management of neuropsychiatric systemic lupus erythematosus (NPSLE) can be challenging due to the complexity of the disease and the lack of specific biomarkers 2, 3.
• The European League Against Rheumatism (EULAR) recommendations for NPSLE provide guidance on diagnosis and treatment, but there is still a need for further research and individualized approaches 4.
• Treatment for NPSLE is often based on the type of neuropsychiatric manifestation, the level of disease activity, and the presence of other risk factors such as antiphospholipid antibodies 2, 3.
• Immunossuppressive therapy, including glucocorticoids and immunosuppressive agents, is commonly used to treat NPSLE, especially in cases where there is evidence of an inflammatory process 2, 4.
• Antithrombotic and anticoagulation therapy may also be recommended for NPSLE manifestations related to antiphospholipid antibodies, such as thrombotic cerebrovascular disease 2, 4.

Current Treatment Approaches

• Hydroxychloroquine is a standard treatment for systemic lupus erythematosus (SLE) and has been shown to reduce disease activity, morbidity, and mortality 5, 6.
• Immunossuppressive agents, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, are often used to treat moderate to severe SLE 5, 6.
• Biologic agents, such as belimumab, rituximab, and anifrolumab, have been approved for the treatment of SLE and may be used in combination with other therapies 5, 6.

Emerging Therapies and Research Needs

• There is a need for further research on the pathogenesis and treatment of NPSLE, including the development of novel biomarkers and therapies 3, 4.
• Emerging therapies, such as biologic agents and small molecule inhibitors, may offer new treatment options for NPSLE and SLE 5, 6.
• Individualized treatment approaches, taking into account the specific needs and characteristics of each patient, are likely to be important in improving outcomes for patients with NPSLE and SLE 2, 3.