How Is Neuropsychiatric Lupus Treated?

Medically Reviewed on 6/7/2022
neuropsychiatric lupus
There are different levels of treatment for neuropsychiatric lupus.

There is no permanent cure for neuropsychiatric systemic lupus erythematosus (NPSLE). Management of NPSLE depends on the severity of symptoms and the underlying cause.

  • Hydroxychloroquine is effective in the long-term treatment of NPSLE. Immunosuppressive medicines help inhibit the activity of the immune system, which can be useful in treating NPSLE.
  • Corticosteroids and other immunosuppressants are effective against active lupus.
  • Blood thinners could be beneficial to treat blood clots, which lead to stroke.
  • Antiseizure medications could effectively control seizures associated with lupus.

There are different levels of treatment for neuropsychiatric lupus. After ruling out other potential causes, and physicians confirming the symptoms are due to systemic lupus erythematosus (SLE), a treatment strategy based on the symptom manifestations and their severity will be implemented.

Treatment strategy for NPSLE

The treatment strategy for NPSLE may include:

  • A general therapeutic approach, such as the treatment of other SLE
  • Primary prevention
  • Management of inflammatory NPSLE
  • Management of cerebrovascular disease

General treatment

General treatment may involve treatment of the symptoms and other alternative therapies. 

Symptomatic treatment or therapy to manage symptoms is the initial step, which is even before confirming the diagnosis. Some of the common medications prescribed to manage the symptoms include:

Cognitive dysfunction, one of the most common symptoms of NPSLE, cannot be easily managed with a symptomatic approach. Hence, alternative therapies, such as counseling or psychotherapy, are recommended to treat symptoms of cognitive dysfunction.

Primary prevention

These are mandatory treatments for SLE. The following two classes of drugs are commonly used to prevent further progression of the disease:

  1. Antimalarial: Hydroxychloroquine and chloroquine are usually prescribed as the first-line treatment for any type of SLE. The treatment with antimalarials for SLE resulted in a reduction in the death rate associated with SLE.
    • Reduce the risk of cerebrovascular disease
    • Reduces the risk of blood clotting
    • Protective effect against seizures
  2. Statins: Statins are known to reduce the inflammatory proteins responsible for neurologic symptoms caused by SLE. The risk of coronary artery disease is higher in people with SLE. Because statins can effectively treat coronary artery disease, they are highly recommended to prevent coronary artery disease associated with SLE.

Management of inflammatory NPSLE

To treat inflammation or prevent clotting associated with NPSLE, the physician may prescribe immunosuppressants, such as corticosteroids, as a single therapy or in combination with other immunosuppressive therapy, such as:

In addition, the physician may recommend the following therapies:

  • Intravenous immunoglobulins involve injecting immunoglobulins to suppress the immune activity in severe cases of SLE
  • Stem cell transplantation to reset the immune system is effective in people who are not responding to other immunosuppressive treatment

Management of cerebrovascular disease

To prevent the formation of blood clots and further development of stroke, the physician may recommend the following drugs:

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Neuropsychiatric lupus treatment chart

Table. Treatment approaches for various symptoms
Symptoms Treatment
Aseptic meningitis (Inflammation of the membranes around the brain)
  • Symptomatic therapy
  • Glucocorticoids (corticosteroids) and immunosuppressive therapy
Cerebrovascular disease
  • Consider thrombolysis
  • High SLE activity or suspicion of brain blood vessel inflammation: Glucocorticoids and immunosuppressive therapy
Demyelinating syndrome (a condition that causes damage to the myelin sheath)
  • Glucocorticoids and immunosuppressive therapy
  • Rituximab if multiple sclerosis overlaps or the diagnosis is not confirmed
Headache
  • Symptomatic therapy
  • Recurrence or association with high SLE activity: Glucocorticoids
Movement disorder
  • Dopamine
  • Tissue death on magnetic resonance imaging (MRI) and acute promyelocytic leukemia (APL) is negative: Antiplatelet therapy
  • Tissue death on MRI and APL found positive: Anticoagulants
  • Normal MRI and APL positive: Antiplatelet therapy or anticoagulants
  • High SLE activity: Adding glucocorticoids and immunosuppressive therapy
Myelopathy (injury to the spinal cord caused by severe compression)
  • Glucocorticoids and immunosuppressive therapy
  • Intense rehabilitation
Seizure disorders
  • First episode: Antiepileptic therapy
  • Recurrence: Chronic antiepileptic therapy
  • Tissue death in MRI and APL found negative: Antiplatelet therapy
  • Tissue death in MRI and APL found positive: Anticoagulants
  • Normal MRI and APL positive: Antiplatelet therapy or anticoagulants
  • High SLE activity: Glucocorticoids and immunosuppressive therapy
Acute confusional state
  • Glucocorticoids and immunosuppressive therapy
Anxiety disorder
  • Psychotherapy
  • Anxiolytics
  • Recurrence or association with high SLE activity: Glucocorticoids
Cognitive dysfunction
  • Psychotherapy and cognitive rehabilitation
  • Tissue death on MRI, APL found positive: Antiplatelet therapy or anticoagulants
  • Progressive or associated with high SLE activity: Glucocorticoids
Mood disorder
  • Psychotherapy
  • Antidepressants
  • Recurrence or association with high SLE activity: Glucocorticoids
Psychosis
  • Antipsychotic agents
  • Associated with high SLE activity: Glucocorticoids and immunosuppressive therapy

How is neuropsychiatric lupus diagnosed?

To diagnose if the neurologic symptoms are caused by systemic lupus erythematosus (SLE), the physicians may use the following techniques to confirm the diagnosis:

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Medically Reviewed on 6/7/2022
References
Image Source: iStock image

Bollu PC. CNS Lupus Workup. Medscape. https://emedicine.medscape.com/article/1146456-workup

Magro-Checa C, Zirkzee EJ, Huizinga TW, Steup-Beekman GM. Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives. Drugs. 2016 Mar;76(4):459-83. doi: 10.1007/s40265-015-0534-3.