Long-term management of JIA
In an accompanying editorial, Jaime Guzman, MD, MSc, and Ross E. Petty, MD, PhD, of British Columbia Children’s Hospital and the University of British Columbia, Vancouver, said a rheumatologist’s interpretation of the study would be tied to what they learned about children with arthritis in medical school. They would see the glass as “half full” if children who achieved remission stayed in remission if they learned that a child might end up outgrowing JIA but potentially develop lifelong disability, whereas others may focus on the outcome of approximately half of patients not achieving remission.
Dr. Jonathan Hausmann
“When I was going through medical school, I remember learning that JIA is a disease of children, and typically, they outgrow it as they become adults,” Dr. Hausmann said. “I think this study and many other studies have shown that that’s actually not the case – that, in fact, it may be a majority of kids continue having active disease even through adulthood.”
If a rheumatologist knows JIA is likely to continue into adulthood, “that’s huge,” Dr. Hausmann said. “That means when we first diagnose patients with JIA as kids, we need to set expectations with the families that this may not just go away; this may be something that could be more lifelong.”
Education on the part of the patient, their parents, and their clinician on the expected trajectory of the disease is critical so that children can continue their own care as they transition to adulthood, Dr. Hausmann explained. “The earlier the kids develop the skills to discuss their medicines, their side effects, the better they’ll be able to transition to adult medicine,” he said.
For the patients who go into remission and stay in remission, the message is also important. “To have the reassurance that a lot of those kids won’t be having active joint symptoms or need to be on medication, that’s a huge positive message that can get out there, so I think that’s great,” Dr. Imundo said.
Time to move on from ILAR classification?
Another big takeaway from the study was how patients’ ILAR classification changed across the 18-year follow-up. First proposed in 1995, the JIA ILAR classification has been revised several times for clarification purposes. In its current form, the ILAR classification considers a patient’s history when categorizing JIA types but also includes factors such as immediate family history. This system of assessing JIA has been criticized and there are initiatives to create a new JIA classification system to replace it.
“The ILAR criteria were designed to classify patients 6 months after disease onset in an attempt to find some commonality in clinical phenotypes, prognosis, and suggested management,” Dr. Wahezi said. “While there continues to be debate as to whether we can improve our classification of JIA patients, it is not surprising that phenotypes may evolve over time as new clinical features develop. As pediatric rheumatologists, we are well accustomed to having to modify management plans as children manifest with new clinical features over time.”
Although the percentage of patients who switched ILAR classifications over the study period was “much higher” than she would have thought, Dr. Imundo said it was the reasons provided in the study that seemed odd to her. “The classification scheme relies on your family history, like someone else in your family now has psoriasis, so your arthritis classification changes,” she explained.
“We want to head toward a much more unified classification scheme, a simpler one. We now understand that some of the diseases that we see in pediatrics are really the equivalent or same disease in adults,” she said.
“Most of the pediatric categories of JIA have distinct adult correlates,” Dr. Hausmann agreed. RF-positive polyarthritis in children and rheumatoid arthritis in adults are correlated, as are systemic JIA and adult-onset Still’s disease, he explained. “That has been borne out also by genetic susceptibility studies that the genetic predispositions to systemic arthritis in children is the same as the genetic predisposition to adult-onset Still’s disease in adults. By and large, there are a lot of similarities between the two.
“I think we need to incorporate some of that knowledge in better classifying kids with JIA so that we can find the best treatments and the best outcomes, and we can provide information to families about the expected course of the disease over time so that can inform our discussions.”
Some pediatric rheumatologists accept the classification system is flawed, but not all concur with the degree to which these problems impact patient care. “While the ILAR classification criteria may be subject to criticism, it does provide general context and prognostic implications for patients and families,” Dr. Wahezi said.
“The medicines certainly are very similar across the JIA categories, so the implications are not as broad” when classification changes,” Dr. Hausmann said. “But it certainly shows that there are things that we still don’t know. I think classification is actually pretty important because it might give you a sense of how persistent the disease will be.”
Dr. Imundo said the ILAR classification’s “time is limited,” and rheumatologists may soon need to adopt a new way of classifying children with rheumatic disease – “a more data-driven, genetics-driven scheme.”
“These categories are so imperfect, and the patients are changing. I feel like that says to me, let’s find something that’s more predictive that really helps us a little better than what we have now,” she said.
The study had no specific funding. The authors of the study and the editorial have disclosed no relevant financial relationships. Dr. Hausmann reports receiving salary support from CARRA. Dr. Imundo and Dr. Wahezi have disclosed no relevant financial relationships.
A version of this article first appeared on Medscape.com.