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Not long ago, pediatric rheumatologists had little in their therapeutic arsenal but aspirin, gold shots and steroids to stave off the ravages of juvenile rheumatoid arthritis (JRA). In exchange for symptom relief, young patients endured growth abnormalities, obesity, high blood pressure, acne and osteoporosis. Today, expert rheumatologic care and advanced therapies at The Children’s Hospital at Montefiore (CHAM) ensure a brighter future for kids with JRA.

Better Therapies Change Treatment Management
“Because we have new agents to treat the disease, we have changed our strategies about how to manage the illness,” says Norman Ilowite, MD, newly appointed chief of Pediatric Rheumatology, CHAM. “We’re less patient – and less willing to accept toxicity from drugs for their efficacy.”

Dr. Ilowite, an internationally recognized JRA expert, led research for the groundbreaking biologic therapeutic etanercept and is the principal investigator for a study of the biologic anakinra – sponsored by the National Institutes of Health (NIH) – that recently showed a 79 percent response rate in patients with systemic JRA, the most severe and therapy-resistant disease subset.

Autoimmune Disorder Affects 300,000 U.S. Children
Juvenile rheumatoid arthritis is an umbrella term used to cover several pediatric inflammatory autoimmune disorders that share arthritis as a symptom. Etiology remains unclear, but JRA’s inflammatory response begins in “a genetically predisposed host,” explains Dr. Ilowite, and “may be triggered by a virus, stress” or other factors. The disease affects about 10 to 20 in 100,000 U.S. children each year.

Distinct Disease Subtypes with A Range of Symptoms
Classification for JRA has recently been reorganized – and renamed – by the International League of Associations of Rheumatology, but most U.S. physicians are familiar with a trifold categorization for the disease. Pauciarticular JRA involves four or fewer joints, often larger joints such as knees. Polyarticular JRA includes five or more joints, usually smaller joints such as those in hands and feet. Systemic JRA is characterized by fever and rash in addition to synovitis – and can also involve organs.

“As medications get better, more and more of our patients benefit. We currently want to put patients in remission with no evidence of active arthritis.”
– Norman Ilowite, MD

Presenting signs and symptoms of JRA include swollen, achy joints, a limp, gait disorders, inflamed eyes and – with systemic disease – spiking fever and a salmon-pink rash. At CHAM, pediatric rheumatologists see a range of JRA complications that include uveitis, flexion contractures, osteoporosis, muscle atrophy, atlanto-axial instability, growth retardation, anemia, organ involvement and, in rare instances, macrophage activation syndrome, a multisystem immune response that can involve liver failure, internal bleeding and a DIC-like coagulopathy.

JRA Patients Often Mistakenly Referred to Orthopedists
Smoldering JRA frequently goes untreated – dismissed by parents as growing pains or misdiagnosed as infection or injury. A study that investigated physicians’ referrals for pauciarticular JRA patients revealed that 63 to 75 percent of examining pediatricians referred pauciarticular JRA patients to orthopedic surgeons – rather than rheumatologists – although patients presented with classic symptoms of synovitis.

Making the JRA Differential
While timely diagnosis and treatment of JRA can prevent or reduce permanent synovial and bone damage, identification of synovitis is not always straightforward. “Physical examinations are probably the most challenging thing we do,” admits Dr. Ilowite.

A careful history and serologic tests can help exclude Lyme disease, parvovirus and strep. Dr. Ilowite always asks patients and parents about “morning preponderance to pain, stiffness associated with inactivity and improvement with exercise,” to rule out growing pains, infection or injury. Lab tests are important to ascertain presence of ANA – associated with uveitis - but are otherwise inconclusive. (For details on disease subset symptoms and lab tests, see “At-a-Glance Rheumatologic Disease”.)

“Higher Goals” for CHAM Patients With JRA
When JRA is suspected, patients benefit from evaluation by a skilled rheumatologic specialist.

CHAM’s pediatric rheumatologists are among the most experienced and accomplished in the world: Dr. Ilowite has treated more than 32,000 children with JRA and other rheumatic disease. He is the principal investigator on an NIH research proposal to study the next-wave JRA biologic IL1-trap – and is committed to exploring the most effective and safe therapeutic options for children with complex rheumatologic disease.

Dr. Ilowite admits he has ambitious goals for his patients. “As medications get better, more and more of our patients benefit,” he explains. “We currently want to put patients in remission with no evidence of active arthritis,” an aim that he believes is “achievable” at CHAM.

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