Dr. Kimura says it is important to stress that these medications help sJIA patients tremendously. She cautions that patients should not stop taking them because of this report.

“It is important we are aware of and look at this issue very carefully. Parents should be cautioned not to stop these medicines just because they are hearing this information and are afraid,” she says. “Stopping these medications can cause sJIA patients to become very ill, including developing something like MAS which can be rapidly fatal. So it would be a big mistake to stop taking medications, and if there are concerns, they should talk to their doctor.”

Dr. Kimura says patients and their parents can keep an eye out for signs and symptoms of these rare complications, which include shortness of breath and difficulty breathing during exercise, especially if this is associated with hard-to-control fevers and a rash, as well as enlarged lymph nodes, an enlarged liver or spleen, or MAS. These symptoms should be reported to the pediatric rheumatologist.

Dr. Kimura says though this data is concerning, this report is helpful because it puts physicians who care for children with sJIA on alert, which in turn should translate to higher detection rates than in the past.

The other important message of this study, says Dr. Kimura, is that parents need to make sure their children are enrolled in registries – such as the CARRA registry – that are attempting to collect information to look for associations like this.

“That is the only way – by collecting information on all patients, whether they take biologics or not – that we will be able to accurately answer the question of whether the incidence of these complications is higher among patients taking certain medications or whether it is because these patients have more severe disease,” Dr. Kimura says.

Deborah McCurdy, MD, a professor of pediatrics and the director of pediatric rheumatology at Mattel Children’s Hospital UCLA, agrees that the CARRA registry is proving to be very helpful in understanding and treating patients with this disease.

“We’re just learning things that we probably weren’t able to realize before because we didn’t have large enough populations to understand all the complications of our diseases,” Dr. McCurdy says.

She also says it’s important to stress to parents that though this is rare, most pediatric rheumatologists are routinely screening for shortness of breath. Still, if you are concerned, she says to mention it to your child’s doctor. Pulmonary hypertension doesn’t develop overnight; it comes on gradually. 

“This is very rare. It’s so rare it isn’t worth losing sleep over,” Dr. McCurdy says. “But if they notice that all the time the child is breathing faster or getting out of breath more than they used to or having trouble climbing stairs or has a dry cough – those are things to mention at the next visit so they can be evaluated.”