Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease where the body’s immune system mistakenly attacks its own healthy tissues. A significant and complex manifestation of SLE is neuropsychiatric lupus (NPSLE), which affects the brain and nervous system.
What is Neuropsychiatric Lupus?
Neuropsychiatric lupus encompasses a wide range of neurological and psychiatric syndromes that occur in individuals with SLE. It can involve both the central nervous system (CNS) and the peripheral nervous system (PNS). NPSLE can manifest even when systemic disease activity is stable or absent, and sometimes, neuropsychiatric symptoms may appear before an SLE diagnosis.
Inflammation plays a role. Autoantibodies can also contribute, with some targeting neural tissues and causing inflammation within the brain. Additionally, issues with blood vessel health, known as vasculopathy, can affect the brain and contribute to symptoms.
Common Manifestations
These manifestations are broadly categorized into neurological, psychiatric, and cognitive domains, and can also involve the peripheral nervous system. Headaches are a very frequent complaint. Seizures, stroke-like events, and movement disorders such as chorea are also observed.
Neurological involvement can also include myelopathy, and cranial neuropathies, affecting specific nerves in the head and face. Psychosis, involving hallucinations or delusions, can occur, along with acute confusional states. These severe manifestations may occur during lupus flares in other organs.
Psychiatric symptoms commonly include mood disorders like depression and anxiety, which are reported in 50% to 60% of SLE patients. Cognitive dysfunction, often referred to as “brain fog,” is a significant concern, affecting 30% to 50% of patients. This can involve issues with memory, difficulty concentrating, impaired executive function (planning and problem-solving), and slowed processing speed. While mild memory problems are common, severe dementia is rare.
The peripheral nervous system can also be affected by NPSLE. This can lead to neuropathies, which are nerve damage causing symptoms like weakness, numbness, or pain in the extremities. Mononeuritis multiplex, a condition involving damage to multiple individual nerves, is another potential peripheral nervous system manifestation.
Diagnosis
Diagnosing NPSLE presents challenges due to the varied and often non-specific nature of its symptoms, which can overlap with other conditions. Many neuropsychiatric symptoms found in SLE patients, such as headaches or mild cognitive dysfunction, can also occur in the general population or be linked to other comorbid conditions like hypertension or diabetes. Therefore, a thorough clinical evaluation is paramount, beginning with a detailed medical history and comprehensive neurological and psychiatric examinations.
Excluding other potential causes for symptoms is a necessary step in the diagnostic process. This involves ruling out infections, medication side effects, or metabolic imbalances, as these can mimic NPSLE manifestations. Imaging studies play a role, with brain MRI scans often used to look for lesions, atrophy, or signs of inflammation within the brain. Magnetic Resonance Angiography (MRA) or Venography (MRV) may also be performed to assess the health of blood vessels.
Cerebrospinal fluid (CSF) analysis, obtained through a lumbar puncture, can provide insights by analyzing the fluid for markers of inflammation or specific antibodies. Neuropsychological testing offers an objective way to assess cognitive function, helping to identify issues with memory, attention, and executive functions. For suspected peripheral nerve involvement, electrophysiological studies like nerve conduction studies and electromyography can help evaluate nerve damage.
Management
The management of NPSLE focuses on reducing inflammation, suppressing the immune system, and alleviating specific symptoms. Treatment approaches are individualized based on the type, severity, and presumed underlying mechanism of the neuropsychiatric event. When symptoms are thought to be due to an inflammatory process, immunosuppressive therapies are often used.
Corticosteroids, such as prednisone, are commonly prescribed for acute flares, with high-dose intravenous methylprednisolone used for severe manifestations like seizures or psychosis. For more severe or chronic cases, other immunosuppressants like cyclophosphamide, azathioprine, or mycophenolate mofetil may be added. Biologic agents, such as rituximab, are considered for refractory disease that does not respond to conventional treatments.
Symptomatic treatment is an important component of NPSLE management. Anticonvulsants are prescribed for seizures, while antidepressants and anxiolytics help manage mood disorders such as depression and anxiety. Antipsychotics are used for psychosis, and pain relievers address headaches or neuropathic pain. When antiphospholipid antibodies are present and associated with thrombotic events, anticoagulation therapy with medications like warfarin is indicated.
Supportive care and lifestyle adjustments also contribute to comprehensive management. Cognitive rehabilitation can help individuals manage cognitive difficulties. Physical therapy may be beneficial for movement disorders or peripheral neuropathies, while psychological counseling can address the emotional burden of the disease. Stress management techniques, ensuring adequate sleep, and maintaining a healthy lifestyle are also encouraged to support overall well-being.