From the Guidelines
Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) manifests clinically with a range of neurological and psychiatric symptoms, including cerebrovascular disease, seizures, severe cognitive dysfunction, major depression, acute confusional state, peripheral nervous disorders, and psychosis, with the most common being cerebrovascular disease and seizures 1. The clinical manifestations of NPSLE can be categorized based on their frequency, with common manifestations (cumulative incidence >5%) including cerebrovascular disease and seizures, and relatively uncommon manifestations (1–5%) including severe cognitive dysfunction, major depression, acute confusional state, peripheral nervous disorders, and psychosis. Some key points to consider in the clinical manifestations of NPSLE include:
- Strong risk factors for NPSLE, such as previous or concurrent severe NPSLE and antiphospholipid antibodies, which increase the risk of certain manifestations like cognitive dysfunction, seizures, and cerebrovascular disease 1
- The importance of a thorough diagnostic work-up to exclude causes unrelated to SLE, including cerebrospinal fluid analysis, EEG, neuropsychological tests, nerve conduction studies, and MRI 1
- The role of glucocorticoids and immunosuppressive therapy in treating NPSLE manifestations thought to reflect an inflammatory process, and antiplatelet/anticoagulation therapy in cases related to antiphospholipid antibodies 1 The diagnosis and treatment of NPSLE should be approached with caution, considering the potential for significant morbidity and mortality, and the importance of early diagnosis and aggressive treatment to prevent permanent neurological damage and improve long-term outcomes. Key considerations in the management of NPSLE include:
- The use of immunosuppressive therapy, such as glucocorticoids, to treat inflammatory manifestations
- The use of antiplatelet/anticoagulation therapy to treat manifestations related to antiphospholipid antibodies
- The importance of regular monitoring of disease activity, medication side effects, and neuropsychiatric symptoms to optimize treatment and prevent complications.
From the Research
Clinical Manifestations of Neuropsychiatric Systemic Lupus Erythematosus (SLE)
The clinical manifestations of Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) are heterogeneous and involve both the central and peripheral nervous system 2. The term NPSLE refers to a series of neurological and psychiatric symptoms directly related to Systemic Lupus Erythematosus (SLE) 3.
Neuropsychiatric Manifestations
Some of the neuropsychiatric manifestations of NPSLE include:
- Neurovascular and psychotic conditions 3
- Mood disorders, psychosis, cognitive impairment, seizures, or headaches 4
- Inflammatory neurologic and psychiatric manifestations 5
Pathogenesis
The pathogenesis of NPSLE is multifactorial and involves various inflammatory cytokines, autoantibodies, and immune complexes resulting in vasculopathic, cytotoxic, and autoantibody-mediated neuronal injury 4. It is also associated with high morbidity and mortality 3.
Diagnosis and Management
The diagnosis and management of NPSLE are complex and challenging due to the lack of a gold standard and the need to exclude other potential causes of neuropsychiatric symptoms 2. Treatment is individualized according to the type of neuropsychiatric manifestations, type of the predominant pathway, activity of SLE, and severity of the clinical manifestations 3.
Treatment Options
Some of the treatment options for NPSLE include:
- High-dose glucocorticoids and intravenous cyclophosphamide for patients with severe symptoms 2
- Rituximab, intravenous immunoglobulins, or plasmapheresis for refractory disease 2, 5, 4
- Azathioprine and mycophenolate for mild to moderate NP manifestations or maintenance therapy 2, 5
- Anticoagulation and antiplatelet agents for symptoms thought to reflect a thrombotic underlying process 2