From the Guidelines
The treatment of neuropsychiatric lupus (NPSLE) should be guided by the most recent EULAR recommendations, which emphasize a tailored approach based on symptom severity and underlying pathophysiology, with hydroxychloroquine and low-dose corticosteroids as first-line treatment for mild to moderate manifestations, and high-dose corticosteroids and immunosuppressants for more severe cases 1.
Key Considerations
- For mild to moderate manifestations like headache, mood disorders, and cognitive dysfunction, first-line treatment typically includes hydroxychloroquine (200-400mg daily) as a foundation therapy, combined with low-dose corticosteroids (prednisone 10-20mg daily) during flares.
- For more severe manifestations such as seizures, psychosis, or cerebrovascular disease, high-dose corticosteroids (methylprednisolone 500-1000mg IV daily for 3-5 days) followed by oral prednisone (1mg/kg/day with gradual taper) are recommended.
- Immunosuppressants are often added for severe or refractory cases, with cyclophosphamide (500-1000mg/m² monthly for 6 months) being the most established option, though mycophenolate mofetil (1-3g daily) or rituximab (375mg/m² weekly for 4 weeks) may be used as alternatives 1.
Symptomatic Treatments
- Symptomatic treatments should be added as needed: antiepileptics for seizures, antipsychotics for psychosis, and antidepressants for mood disorders.
- Anticoagulation with warfarin (target INR 2-3) or direct oral anticoagulants is indicated when antiphospholipid antibodies are present with thrombotic events.
Monitoring and Adjustments
- Regular monitoring of disease activity, medication side effects, and neuropsychiatric symptoms is essential, with treatment adjustments made accordingly.
- The updated EULAR recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion 1.
From the FDA Drug Label
1 INDICATIONS AND USAGE
- 3 Systemic Lupus Erythematosus Hydroxychloroquine sulfate tablets are indicated for the treatment of systemic lupus erythematosus in adults.
The treatment of Neuropsychiatric Lupus is not directly addressed in the provided drug label.
- The label mentions Systemic Lupus Erythematosus as an indication for hydroxychloroquine sulfate tablets, but it does not specifically mention Neuropsychiatric Lupus.
- Therefore, no conclusion can be drawn about the treatment of Neuropsychiatric Lupus with hydroxychloroquine sulfate tablets based on the provided label 2.
From the Research
Treatment Options for Neuropsychiatric Lupus
- The management of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is multimodal and includes nonsteroidal anti-inflammatory drugs, anticoagulation, and immunosuppressives such as cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate 3.
- For refractory NPSLE, intravenous immunoglobulin (IVIG), plasmapheresis, and rituximab have been used 3, 4.
- Adjunctive symptomatic treatment complements these therapies by targeting mood disorders, psychosis, cognitive impairment, seizures or headaches 3.
- Immunosuppressants are typically indicated in recurrent seizures, optic neuritis, myelopathy, psychosis and peripheral nerve disease, although a low threshold is recommended for cerebrovascular disease and other NP manifestations, especially when SLE is active 4.
- Anti-coagulation is rarely indicated in other syndromes, with the exception of stroke with positive antiphospholipid antibodies 4.
Pharmacologic Management
- The mainstay of therapy relies on immune-suppression by glucocorticoids, in adjunction with cyclophosphamide or anti-B-cell therapy, in moderate to severe cases 5.
- In selected scenarios (e.g., chorea) intravenous immunoglobulin or plasmapheresis may be effective 5.
- Anticoagulation is warranted if anti-phospholipid antibodies are present 5.
- Nonsteroidal anti-inflammatory drugs (NSAID) are commonly used to treat musculoskeletal symptoms, serositis and headache in SLE patients, but require awareness of potential side effects and close monitoring of toxicity 6.
Future Perspectives
- Advances in the fields of biomarkers, neuroimaging for brain structural, perfusion or functional abnormalities, and design of novel compounds targeting not only systemic autoimmunity but also inflammatory and regenerative pathways within the nervous system, hold promise for optimizing NPSLE management 4.
- Several new biological agents are being tested, including Belimumab, a human monoclonal antibody that targets B lymphocyte stimulator 3.