Children Remain High Risk for Antiphospholipid Syndrome

The initial high-risk antibody for antiphospholipid syndrome (APS) has remained high in pediatric individuals, according to research conducted by investigators from the Hospital for Special Surgery (HSS).

APS is defined as a rare autoimmune disorder that occurs when antibodies made by the immune system— antiphospholipid antibodies (aPL), attack proteins that bind the phospholipid cell walls of blood cells that are in the inner layer of arteries and veins. The condition could increase the risk of dangerous blood clots inside blood vessels, according to the study authors. The increase could lead to strokes, heart attacks, and issues among pregnant individuals.

“There are a lot of unanswered questions about how APS affects pediatric patients since evidence to date has been very limited,” said Jheel Pandya, MD, a pediatric rheumatology fellow at HSS and lead author of the research, in a press release. “Our study reveals that an initial high-risk antiphospholipid antibody profile is unlikely to be transient in pediatric patients, indicating their risk of serious health problems remains elevated and they should be followed carefully.”

A patient that has experienced symptoms is then tested by rheumatologists using 3 laboratory tests to assess the incidence of aPL in the body. The tests include a lupus anticoagulant (LA), an anticardiolipin antibody (aCL) and an anti-beta-2-glycoprotein-1 antibody (aβ2GPI). However, these tests are not yet classified for pediatric individuals.

In the study, Pandya and investigators examined electronic medical records for individuals aged 10-18 that were aPL positive. The individuals were then placed in groups of high and low risk, based on the initial positive result. The groups were divided by individuals that had a positive LA test, and/or a higher aCL level and aβ2GPI antibodies, were placed as high-risk and individuals that had a negative LA test and/or a lower aCL level and aβ2GPI antibodies, were placed as low risk.

The researchers found that 93% of individuals that were in the higher risk group had persistently positive PL compared to 47% of individuals that were in the lower risk group.

“The more we can learn about differences in APS development and progression in pediatric patients compared with adults, the more we can optimize their diagnosis and treatment,” said Karen Brandt Onel, MD, chief of Pediatric Rheumatology at HSS, co-author, in a press release. “This study underscores the importance of continuing to follow pediatric patients with higher-risk profiles and transitioning them to adult care after the age of 18.”

Other findings included that 32 individuals remained positive over time and 26 individuals did not have persistently positive results— in either group. However, the among the individuals that remained positive in the high-risk group, 9 experienced blood clots, compared to none in the low-risk group. Among the 26 individuals, none experienced blood clots, but 1 reported a skin rash, and 2 reported migraines.

“Our results highlight the need for a large-scale, international effort to better understand how APS affects pediatric patients,” said Doruk Erkan, MD, MPH, a physician-scientist at the Barbara Volker Center for Women and Rheumatic Diseases, attending rheumatologist at HSS and professor of medicine at Weill Cornell Medicine, senior author, in a press release. “An international effort has been initiated to pool pediatric APS data and ultimately guide the development of classification criteria for pediatric patients.”

Reference

A high-risk antiphospholipid antibody profile matters in pediatric patients with antiphospholipid syndrome. EurekAlert!. News release. November 12, 2023. Accessed November 17, 2023. https://www.eurekalert.org/news-releases/1007667.