WASHINGTON, D.C.—Addressing a gathering of healthcare providers at the 2016 ACR/ARHP Annual Meeting concurrent session titled, Pediatric Rheumatology for the Adult Rheumatologist, part of the ACR Review Course, expert Sangeeta Sule, MD, PhD, associate professor of pediatrics specializing in rheumatology at Johns Hopkins Hospital in Baltimore, displayed a color-coded map of the U.S. on which purple-shaded states represented regions without pediatric rheumatologists. There were several purple states.
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“There’s a national shortage of pediatric rheumatologists,” Dr. Sule said, using the visual aid to make clear the need for more physicians in the specialty.
It stands to reason, then, that adult rheumatologists either already are treating children or will need to do so as this shortage worsens. With this in mind, she gave an overview of rheumatologic issues faced by children and pointed out how they differ from those that affect adults.
Pediatric Inflammatory Conditions
Inflammatory conditions in children include Henoch-Schönlein purpura, which most commonly presents as a purpuric rash, starting in the leg and proceeding up to the trunk. It accounts for 49% of all pediatric vasculitis, has a peak onset at 4–6 years old, is seen more often in boys and is more common in winter and spring. Treatment includes supportive care, hospitalization in severe cases, and urinalysis and blood pressure monitoring every week or two for the first couple of months.
“Our goal as pediatricians and pediatric rheumatologists is [to] support these children while minimizing complications,” Dr. Sule said.
In Kawasaki disease, children have fever for at least five straight days, along with other factors, such as bloodshot eyes and oral mucous membrane changes, including “strawberry tongue,” Dr. Sule noted. These children typically are treated with intravenous immunoglobulin, which can reduce the incidence of coronary artery aneurysms in these patients.
In neonatal lupus, which can result in such complications as rash, heart block and hepatitis, the main goal is to prevent heart block, Dr. Sule said. Pregnant women with anti-Ro or anti-La antibodies should have a Doppler fetal echocardiogram every week from weeks 18 to 26, then every other week through 32 weeks. If second-degree heart block is found, fluorinated glucocorticoids are called for, she said. Pre-emptive treatment with hydroxychloroquine has been shown to be safe and effective, she added. Prevention is the goal, since third-degree heart block can’t be corrected medically and will require a pacemaker.
Non-Inflammatory Pediatric Conditions
Dr. Sule also identified several non-inflammatory pediatric rheumatic conditions, such as:
- Hypermobility, which is characterized by intermittent muscular pain not related to the joints;
- Legg-Calve-Perthes disease, or idiopathic osteonecrosis of the hip, which requires an X-ray to determine whether it’s bilateral;
- Slipped capital femoral epiphysis, which is a hip condition in which the femur head slips off the body of the femur. It is most commonly seen in overweight boys and is often bilateral; and
- Osgood-Schlatter disease, which involves pain and swelling at the tibial tuberosity that is worsened by activity and mostly treated with supportive care.
Speaking on the topic of arthritis in children, Dr. Sule noted that most cases of juvenile idiopathic arthritis (JIA) are oligoarticular, affecting up to four joints in the first six months. It tends to involve the large joints—knees, ankles, wrists and elbows—and can be seen nearly throughout childhood. Girls are affected more frequently than boys.
“It’s a very subtle presentation,” Dr. Sule said. Often, the disease manifests itself with the child wanting to be picked up just after waking up in the morning and after naps, because they’re stiff, she noted.
The polyarticular form affects girls more than boys by a three-to-one margin and is usually seen when children are either very young or in their teens. It tends to affect the knees, ankles, wrists and hands. Dr. Sule said it’s important for clinicians to monitor a child’s growth, in addition to their joints.
Systemic JIA can present at any age and is equally prevalent in girls and boys. It includes a fleeting, salmon-colored rash, which can come and go with fever. “If you don’t see it [the rash] the first time, go back and back and back,” she said.
‘It’s very important to start this process early, to start talking to children & their parents about this planned process of transition from as young as ages 11 to 14.’ —Dr. Sule
The disease typically involves a “quotidian fever,” that is, a fever spike followed by hypothermia once or twice a day. This pattern is not typically seen in other causes of fever, so it makes sense to keep track of this, Dr. Sule said.
Anterior uveitis is a particularly important issue to monitor in JIA, especially because it involves no symptoms.
“It’s very important to recognize that these children need to be screened,” she said. In oligo- and polyarthritis JIA, screening for anterior uveitis should be done every three to four months through age 7, then every four to six months. Yearly screenings are acceptable in systemic JIA, since these children are not at an increased risk.
One key difference between children with JIA and adults with rheumatoid arthritis is that children tend to be able to live disease free without treatment much more commonly than adults, Dr. Sule said.
Dr. Sule also stressed the importance of transitioning children from their pediatric rheumatologist to an adult rheumatologist over a period of time, rather than all at once, so the young adult can become versed in their disease and medications and can develop self-management skills. It’s helpful for the pediatric rheumatologist to prepare a medical summary for the adult rheumatologist, she said. “Get acquainted” visits with the adult physician can be helpful as well.
“It’s very important to start this process early, to start talking to children and their parents about this planned process of transition from as young as ages 11 to 14,” Dr. Sule said, “so that it’s a process of development—not just that they turn 21 and at their last visit they’re seen by the pediatric rheumatologist and told to find an adult rheumatologist.”
Thomas R. Collins is a freelance writer living in South Florida.