THE OFFICIAL NEWS SOURCE OF ACR CONVERGENCE 2022 • NOVEMBER 10-14



Pediatric rheumatologists stress importance of early recognition of juvenile localized scleroderma

Suzanne Li, MD, PhD
Suzanne Li, MD, PhD

Recognizing active localized scleroderma in pediatric patients is not only important for diagnosing the problem early, it’s also important for knowing when treatment is necessary and when it can be stopped. 


During a Tuesday session, two experts will review the latest evidence and offer practical strategies for clinicians during Juvenile Localized Scleroderma: Does this Child Have Active Disease?, which will take place from 11:00 am – 12:00 pm in Room B216-B217, Building B in the Georgia World Congress Center. 


Professor Suzanne Li, MD, PhD, a pediatric rheumatologist at Hackensack University Medical Center, said one of the session’s primary goals was to raise awareness of the issues related to juvenile localized scleroderma, which can be more serious than many rheumatologists realize and often is diagnosed late. 


“There is a great need to recognize when to treat this disease, because, as with many of our other rheumatic diseases, there’s a window of opportunity,” Dr. Li said. “The treatment that we currently have is for the active inflammatory stage, as is typical for many of our diseases. The problem with localized scleroderma is that it’s really affecting more than the skin. Kids tend to have worse outcomes compared to patients with adult-onset disease because they have a much higher incidence of deep tissue and extracutaneous involvement, and much longer disease duration. Most of the kids end up with damage, and because the disease spans childhood, they have problems such as hemiatrophy of their face, a limb, or parts of their trunk such as the breast.”


She hopes that by spreading awareness of the need for early treatment, these issues can be avoided or limited. 


“If we don’t recognize it, the child often ends up with a severe deformity or disability,” she said. “Even when they’re treated, more than 25% of pediatric patients have functional impairment, so we’ve clearly not optimized care even when we’re recognizing the disease.” 


During her talk, “Assessment of Disease Activity in Localized Scleroderma,” Dr. Li will help attendees improve their ability to recognize active disease. 


“What makes localized scleroderma harder to recognize is that, unlike diseases such as juvenile idiopathic arthritis, kids usually don’t have symptoms early,” she said. “With localized scleroderma, it doesn’t hurt early on. If there’s a skin lesion, it usually doesn’t bother the child much and, because it’s rare, people may not recognize what it is or that it’s something serious.” 


To help clinicians, Dr. Li will review the latest research looking at features — both cutaneous and extracutaneous — associated with disease activity for juvenile localized scleroderma. 


“With the cutaneous symptoms, one key point is there really is no universal activity feature,” she said. “We’ve found there’s no feature common to all active lesions, so that means you have to assess several features to tell if a patient has activity. You can’t just rely on erythema, which has been considered to be a key activity feature.


“Another feature frequently used is skin thickness, which is present in all scoring measures for localized scleroderma. But we found it’s not specific to active lesions. Even looking at changes in skin thickness over time didn’t differentiate between active and inactive lesions. So again, we need to assess several features to tell if a patient has active disease.” 


When it comes to extracutaneous symptoms, it gets even trickier, Dr. Li said. 


“There often isn’t a great correlation between the extra-cutaneous and the cutaneous symptoms,” she said. “Clinicians need be aware that there may be a disconnect, so they need to evaluate both types of involvement to decide upon treatment.” 


Accurate extracutaneous assessment often requires imaging or other evaluations, which Kathryn Torok, MD, Associate Professor of Pediatrics at the University of Pittsburgh, Pennsylvania, will discuss in her talk “Additional Tools to Evaluate Disease Status in Localized Scleroderma.”


Dr. Torok will review imaging modalities for cutaneous and extra-cutaneous assessment as well as other tools with potential clinical utility to monitor disease status. She will also discuss the standard assessment for patients with localized scleroderma involving the head.