Why Childhood SLE is More Severe than Adult SLE Save

Hi everyone. My name is Sangeeta Sule. I am the division director of pediatric rheumatology at Children's National Hospital in Washington, DC. I'm here today to talk about why pediatric lupus patients have more severe lupus than adult patients.

First off, let's just talk about pediatric lupus.

About 10 to 20% of all lupus patients are children. So it can happen, and, I want to stress that children are not miniature adults. All of us know that, but sometimes it takes a little bit for our science to catch up with that.

What I mean by that is that the criteria to make the diagnosis of lupus are the same in pediatrics and adults.

The main difference is that the pediatric population is defined as those less than 18. In other words, a pediatric patient would be someone who has lupus diagnosed before age 18.

The average age of lupus in the pediatric population is around 11 to 12 years old, but it can occur at any age. It can occur pre-pubertal and post-pubertal. The main difference between these two are the factors associated with the lupus. So for example, in the pre-pubertal population, the female-to-male ratio is about four-to-one, whereas in the post-pubertal population, it's about seven-to-one ratio. Compare that to the adults, where the female to male ratio is about nine to 10 to one, and you can see across all ages there's a female predominance, but slightly less in the pre-pubertal population.

The other thing that's different between pediatric lupus and adult lupus is why children develop lupus in the first place. Especially in the very young age-of-onset group, there's thought to be - probably - a hormonal play (meaning exposure to estrogen or progesterone may increase the risk) and/or an immune system imbalance, such as immune dysregulation. For example, complement deficiencies are associated with increased prevalence of lupus in the childhood age.

Just like in adults, childhood lupus is more common in Asians, African-American and Hispanic populations.

One of the things I want to stress in this video is that pediatric patients can present very abruptly and very acutely with multi-organ involvement, which can be quite different from the adult presentation.

Children can present upfront with severe manifestations, including hematologic abnormalities, nephritis, and CNS manifestations. These CNS manifestations are usually quite abrupt and include things like encephalopathy, compared to the adult manifestations, which may include more cranial nerve abnormalities.

Adults also have an increased prevalence of sicca symptoms and pleuritis compared to the pediatric populations. As you can imagine, because of this discrepancy between the pediatric and adult lupus patients, the pediatric patients are also needing to be treated aggressively and usually with higher doses of steroids upfront compared to the adult population to maintain disease control.

So multiple studies have shown that the exposure across the age spectrum of steroids is higher in the pediatric population compared to the adults.

This means, of course, that morbidity from both the lupus and the treatments that we're using in lupus, are higher in pediatric populations. Pediatric lupus patients are at increased risk for complications from steroids, such as diabetes, avascular necrosis, osteoporosis. Things like cardiovascular disease, which are increased in the adult population, may not occur as frequently or may not be recognized while children are children suffering from lupus, but they may present with adult-onset cardiovascular disease at a younger age than would be anticipated.

One of the other unique factors for pediatric lupus patients is exposure to steroids may affect things that we wouldn't expect in adults, such as growth or long-term development. So for example, children with lupus are often shorter than their age-match peers without disease because of exposure to the disease itself, the increased inflammatory component, and medications such as steroids.

If you want to find out more about pediatric lupus, please check out RheumNow.com and just remember that kids are not little adults and that pediatric lupus patients can present with aggressive disease, and it's important to recognize and treat them aggressively. Thank you.