Lupus is an autoimmune disease that can affect many parts of the body, including the brain. Lupus causes inflammation which can injure tissues and organs. When lupus affects the brain, it is called neuropsychiatric systemic lupus erythematosus or NPSLE. About 40-70% of people with lupus experience some neurological or psychiatric symptoms. These nervous system problems can sometimes be severe and life-threatening. Understanding how lupus impacts the brain and being able to recognize symptoms of NPSLE is important for getting proper treatment.
What are common neurological symptoms of lupus?
Some of the most common neurological manifestations of lupus include:
– Headaches – These are often severe and debilitating headaches that can mimic migraines. Headaches are one of the most frequently reported neurological symptoms, affecting about 40% of lupus patients.
– Cognitive dysfunction – This involves problems with memory, concentration, reasoning, and other cognitive skills. Around 40% of lupus patients experience mild cognitive impairment. It can be one of the early symptoms of NPSLE.
– Seizures – About 10-20% of lupus patients have seizures. These may be generalized seizures affecting the whole brain or partial seizures that impact a localized area of the brain. Seizures often indicate active lupus.
– Stroke – People with lupus have a significantly increased risk of stroke compared to the general population. Stroke risk is 2 to 10 times higher. Strokes are more likely in people who have antiphospholipid antibodies.
– Demyelinating syndrome – This rare complication damages the protective covering around nerves in the brain and spinal cord. It can cause visual loss, weakness, numbness and other neurological symptoms.
What psychiatric symptoms are associated with lupus?
Neuropsychiatric lupus can also lead to various behavioral and mental health issues, including:
– Anxiety disorder – Estimates suggest around 30% of lupus patients have some form of anxiety disorder.
– Depression – Up to 40% of lupus patients experience clinical depression. This may be a reaction to living with a chronic illness or due to lupus affecting brain function.
– Psychosis – A small percentage of lupus patients develop psychotic symptoms like hallucinations, delusions, and disordered thinking. This is more common during active systemic lupus.
– Cognitive dysfunction – As mentioned previously, many lupus patients have trouble with memory, learning, attention, and other cognitive skills.
– Mood disorders – Irritability, emotional instability, uncontrolled laughter or crying, and suicide attempts are psychiatric manifestations of lupus. About 20% of lupus patients have mood disorders.
What causes neuropsychiatric symptoms in lupus patients?
Doctors do not fully understand why lupus affects the nervous system, but several possible causes include:
– Autoantibody activity – Antibodies that attack the body’s own healthy tissues can cross the blood-brain barrier and bind to brain cells, causing neurological injury.
– Blood vessel damage – Lupus can cause blood clots and bleeding in the brain due to vascular damage. This disrupts normal blood flow.
– Blood-brain barrier disruption – Lupus may damage the blood-brain barrier that normally protects the brain and spinal cord. This allows harmful immune cells and proteins into the central nervous system.
– Immune complex deposition – Immune complexes (clusters of autoantibodies bound to antigens) can get deposited in the blood vessels and tissues of the brain. This triggers damaging local inflammation.
– Infection – Having lupus appears to increase the risk of certain infections, like human herpesvirus-6, that in turn can provoke neurological and psychiatric problems.
– Medication side effects – Some medications used to treat lupus, like corticosteroids, can cause psychiatric symptoms as side effects.
How is neuropsychiatric lupus diagnosed?
Diagnosing NPSLE can be challenging because there are no definitive tests. Doctors will:
– Evaluate symptoms – Asking about neurological and psychiatric symptoms and doing a physical exam provides initial clues.
– Review medical history – Looking at past lupus symptoms helps determine if neuropsychiatric issues are new or have occurred before.
– Run blood tests – Blood tests help detect antibodies associated with NPSLE and look for other abnormalities.
– Analyze spinal fluid – A lumbar puncture can show elevated protein or white blood cell levels in the cerebrospinal fluid.
– Use neuroimaging – MRI, CT, or PET scans of the brain can reveal areas of inflammation, bleeding or ischemia.
– Assess cognitive function – Formal neurocognitive testing can identify deficits in memory, attention, reasoning, etc.
– Rule out other causes – Doctors must exclude other possible causes like infections, medication side effects, or neurological disorders.
What are the treatment options for NPSLE?
Treatments focus on relieving acute symptoms and preventing recurrence. Options may include:
– Corticosteroids – Steroids like prednisone reduce inflammation and are often the first-line treatment. High doses are used for major symptoms.
– Immunosuppressants – Drugs like azathioprine, mycophenolate, and methotrexate help suppress the overactive immune system.
– Plasmapheresis – This blood filtration technique removes autoantibodies from the blood circulation. It quickly stabilizes severe neurological manifestations.
– IVIG – Intravenous immunoglobulin provides healthy antibodies to block autoantibody activity. It may help reverse psychosis and cognitive impairment.
– Plasma exchange – This removes abnormal immune components from the blood using a centrifuge. It provides rapid stabilization but benefits are usually temporary.
– Rituximab – This biologic drug depletes B cells involved in harmful autoantibody production. It may benefit patients with refractory neuropsychiatric lupus.
– Cyclophosphamide – This potent immunosuppressant can treat severe, rapidly progressive NPSLE that doesn’t respond to other therapies.
– Anticoagulants – Blood thinners like warfarin can help prevent clotting and stroke from antiphospholipid antibodies.
What is the prognosis for neuropsychiatric lupus?
The outlook for NPSLE depends on the severity of symptoms. Milder neurocognitive dysfunction may not progress with proper control of systemic lupus activity. Severe manifestations like stroke or psychosis can lead to irreversible damage and long-term disability. However, early detection and aggressive treatment of flares can help prevent permanent impairment in many patients.
About 60-80% of people who develop major neuropsychiatric problems related to lupus will recover fully or have mild residual symptoms once the flare resolves. The remainder have ongoing cognitive dysfunction or disability. Overall, the presence of certain neuropsychiatric symptoms is linked to a higher mortality risk. Close follow up and monitoring is essential for patients with lupus affecting the nervous system.
Lifestyle measures to help manage neuropsychiatric lupus
Besides medical treatment, some lifestyle strategies can help minimize symptoms:
– Get adequate rest and sleep to give your brain a chance to recover. Stress can exacerbate neurological problems.
– Stick to a regular sleep schedule and address any sleep disorders like sleep apnea. Quality sleep is vital for brain health.
– Exercise regularly to improve circulation and oxygenation to the brain. Even mild exercise like walking helps.
– Do mental exercises like crossword puzzles or learning a new language to keep your brain active and engaged.
– Avoid smoking and limit alcohol intake, as these can worsen neurological manifestations and make medications less effective.
– Use stress management techniques such as meditation, yoga, or counseling to cope with anxiety, depression and emotional difficulties.
– Get proper treatment for any metabolic disorders like high blood pressure, diabetes or high cholesterol that increase risks of stroke and cognitive decline.
– Ask your doctor about taking a low dose aspirin to inhibit clotting if you have antiphospholipid antibodies and high stroke risk.
Key takeaways
– Up to 70% of lupus patients experience some type of neurological or psychiatric disorder, referred to as NPSLE.
– Headaches, cognitive impairment, mood disorders and psychosis are among the common neuropsychiatric manifestations.
– Autoantibody activity, blood vessel damage, infection and medications may trigger these nervous system issues.
– Diagnosing NPSLE involves evaluating symptoms, blood tests, spinal fluid analysis and neuroimaging.
– Treatments include steroids, immunosuppressants, plasmapheresis, IVIG and monoclonal antibodies.
– Mild cases can resolve fully with treatment but severe manifestations may cause irreversible damage.
– Lifestyle measures like getting adequate rest, exercising, and managing stress can help improve neurological outcomes.
