Although 30–50% of patients diagnosed with pediatric systemic lupus erythematosus (pSLE) experience neuropsychiatric manifestations of the disease (NPSLE), a poor understanding of the underlying pathophysiology remains, creating challenges for disease management. Children with NPSLE can experience a range of syndromes, such as cognitive impairment, psychiatric disorders, seizures and strokes, which are often treated with broad immunosuppressive medications. These medications may cause significant side effects, such as growth delay and infections.
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“In clinical practice, there are still many unknowns about how lupus affects the brain and how this may affect a child’s developing brain,” says Andrea Knight, MD, MSCE, a rheumatologist who specializes in managing pSLE and other pediatric rheumatic diseases at The Children’s Hospital of Philadelphia in Pennsylvania.
Seeing a Clearer Picture
Dr. Knight believes that combining information provided by different neuroimaging modalities, such as structural magnetic resonance imaging (sMRI), diffusion tensor imaging (DTI) and functional MRI (fMRI), may allow enhanced characterization of underlying neural mechanisms to better detect the earliest signs of NPSLE.
This research question stems from her previous work using neuroimaging to investigate neurologic structural changes in patients with pSLE. In 2016, Dr. Knight presented the initial findings from her research using sMRI to compare brain volumes in pediatric lupus patients with control patients at the 2016 ACR/ARHP Annual Meeting.1
“Overall, brain volume did not differ. However, gray matter in the regions of the brain known to control cognitive and emotional function was smaller in the lupus patients, suggestive of damage,” she explains. These changes were present early in the disease course, which averaged about a year in the lupus group. Additionally, the initial signs of damage in the same brain regions for a subgroup of pediatric lupus patients without known neuropsychiatric impairment were also present, signifying potential subclinical effects of pSLE on the brain.
By drawing on the expertise from psychiatry and neurology to understand how the brain is affected by inflammatory disorders, Dr. Knight believes combining the unique views of the brain seen through sMRI to study brain structure, fMRI to see brain activity and DTI to see neural tract pathways may uncover a more telling story of how pSLE affects brain regions, their connections and function.
“By combining the data from these modalities, we can get very specific information on brain regions, volume and, if injury has occurred, how the integrity of brain tissue and brain activity between different regions has been affected,” she says.