Review of Neuropsychiatric Lupus Diagnosis and Management

Neuropsychiatricsystemic lupus erythematosus (NPSLE), or central nervous system lupus, can be challenging to diagnose because of the variability in how it manifests itself in different people. In a new study, researchers reviewed recent advances in NPSLE from a clinical perspective, with specific focus on epidemiology of the disease, clinical presentation, diagnostic evaluation, and treatment. 

In this research review, researchers synthesized and summarized existing research. Although outcomes for NPSLE are generally favorable, the condition significantly negatively impacts health-related quality of life, imposes a considerable economic burden, and is associated with an increased risk of mortality. A few key findings from the review include: 

• Clinical Presentation
  • NPSLE can present with a wide range of manifestations, including cognitive dysfunction, mood disorders, psychosis, seizures, headaches, and cerebrovascular events among others. The variability, along with the overlap of NPSLE symptoms with other neurological and psychiatric disorders, makes it hard to pinpoint a diagnosis.
  • There is also interplay between inflammatory and vascular mechanisms which add to the diagnostic challenge. 
• Diagnostic Evaluation
  • There is a lack of specific diagnostic tests for NPSLE. Diagnosis often relies on a combination of clinical evaluation, laboratory tests, and neuroimaging tests (MRI and PET scans).
  • Numerous autoantibodies , biomarkers, and neuroimaging markers have been reported, but further investigation is needed for their potential role in diagnosis and monitoring disease activity. 
• Treatment / Therapeutic Options
  • Treatment is dependent on the individual’s genetic, immunological and clinical characteristics. Severity and suspected pathogenesis play a role in determining a person’s therapeutic options.
  • Immunosuppressive therapies (corticosteroids) are the main treatments for NPSLE. Biologic therapies like rituximab and belimumab are showing positive outcomes in refractory cases, but it is still unclear with limited data. 

While there has been significant progress made in understanding and managing NPSLE, further research and clinical studies for NPSLE are needed to better inform management of the disease. Learn more about lupus and the nervous system.

Read the study